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1. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

Author: Bütikofer L, Varisco PA, Distler O, Kowal-Bielecka O, Allanore Y, Riemekasten G, Villiger PM, Adler S, Avouac J, Walker UA, Guiducci S, Airò P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Gurman AB, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Müller-Ladner U, Maurer B, Tyndall A, Lapadula G, Iannone F, Becvar R, Sierakowsky S, Bielecka OK, Cutolo M, Sulli A, Cuomo G, Vettori S, Rednic S, Nicoara I, Vlachoyiannopoulos P, Montecucco C, Caporali R, Novak S, Czirják L, Varju C, Chizzolini C, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Rozman B, Mallia C, Coleiro B, Gabrielli A, Farge D, Hij A, Hesselstrand R, Scheja A, Wollheim F, Martinovic D, Govoni M, Monaco AL, Hunzelmann N, Pellerito R, Bambara LM, Caramaschi P, Black C, Denton C, Henes J, Santamaria VO, Heitmann S, Krasowska D, Seidel M, Oleszowsky M, Burkhardt H, Himsel A, Salvador MJ, Stamenkovic B, Stankovic A, Tikly M, Starovoytova MN, Engelhart M, Strauss G, Nielsen H, Damgaard K, Szücs G, Mendoza AZ, de la Puente Buijdos C, Sifuentes Giraldo WA, Midtvedt Ø, Garen T, Launay D, Valesini G, Riccieri V, Ionescu RM, Opris D, Groseanu L, Wigley FM, Mihai CM, Cornateanu RS, Ionitescu R, Gherghe AM, Gorga M, Dobrota R, Bojinca M, Schett G, Distler JHW, Meroni P, Zeni S, Mouthon L, De Keyser F, Smith V, Cantatore FP, Corrado A, Ullman S, Iversen L, Pozzi MR, Eyerich K, Hein R, Knott E, Szechinski J, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Krummel-Lorenz B, Saar P, Aringer M, Günther C, Anic B, Baresic M, Mayer M, Radominski SC, de Souza Müller C, Azevedo VF, Agachi S, Groppa L, Chiaburu L, Russu E, Zenone T, Stebbings S, Highton J, Stamp L, Chapman P, Baron M, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Rosato E, Pisarri S, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Chirieac R, Ancuta C, Furst DE, Kafaja S, de la Peña Lefebvre PG, Rubio SR, Exposito MV, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Venalis A, Butrimiene I, Venalis P, Rugiene R, Karpec D, Kerzberg E, Montoya F, Cosentino V, Castellvi I., Publica, Bütikofer, L, Varisco, Pa, Distler, O, Kowal-Bielecka, O, Allanore, Y, Riemekasten, G, Villiger, Pm, Adler, S, Avouac, J, Walker, Ua, Guiducci, S, Airò, P, Hachulla, E, Valentini, G, Carreira, Pe, Cozzi, F, Gurman, Ab, Braun-Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller-Ladner, U, Maurer, B, Tyndall, A, Lapadula, G, Iannone, F, Becvar, R, Sierakowsky, S, Bielecka, Ok, Cutolo, M, Sulli, A, Cuomo, G, Vettori, S, Rednic, S, Nicoara, I, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Novak, S, Czirják, L, Varju, C, Chizzolini, C, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Hij, A, Hesselstrand, R, Scheja, A, Wollheim, F, Martinovic, D, Govoni, M, Monaco, Al, Hunzelmann, N, Pellerito, R, Bambara, Lm, Caramaschi, P, Black, C, Denton, C, Henes, J, Santamaria, Vo, Heitmann, S, Krasowska, D, Seidel, M, Oleszowsky, M, Burkhardt, H, Himsel, A, Salvador, Mj, Stamenkovic, B, Stankovic, A, Tikly, M, Starovoytova, Mn, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szücs, G, Mendoza, Az, de la Puente Buijdos, C, Sifuentes Giraldo, Wa, Midtvedt, Ø, Garen, T, Launay, D, Valesini, G, Riccieri, V, Ionescu, Rm, Opris, D, Groseanu, L, Wigley, Fm, Mihai, Cm, Cornateanu, R, Ionitescu, R, Gherghe, Am, Gorga, M, Dobrota, R, Bojinca, M, Schett, G, Distler, Jhw, Meroni, P, Zeni, S, Mouthon, L, De Keyser, F, Smith, V, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, Pozzi, Mr, Eyerich, K, Hein, R, Knott, E, Szechinski, J, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Krummel-Lorenz, B, Saar, P, Aringer, M, Günther, C, Anic, B, Baresic, M, Mayer, M, Radominski, Sc, de Souza Müller, C, Azevedo, Vf, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Zenone, T, Stebbings, S, Highton, J, Stamp, L, Chapman, P, Baron, M, O'Donnell, J, Solanki, K, Doube, A, Veale, D, O'Rourke, M, Loyo, E, Rosato, E, Pisarri, S, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Chirieac, R, Ancuta, C, Furst, De, Kafaja, S, de la Peña Lefebvre, Pg, Rubio, Sr, Exposito, Mv, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Kerzberg, E, Montoya, F, Cosentino, V, and Castellvi, I.
Subjects: INVOLVEMENT, Male, Hypertension, Renal, ACE inhibitors, lcsh:Diseases of the musculoskeletal system, Scleroderma Renal Crisis, MULTICENTER, Angiotensin-Converting Enzyme Inhibitors, Scleroderma, Scleroderma renal crisis, 0302 clinical medicine, Risk Factors, Medicine and Health Sciences, Medicine, 030212 general & internal medicine, Prospective Studies, 610 Medicine & health, Renal, Antihypertensive drugs, Outcome, antihypertensive drugs, arterial hypertension, outcome, scleroderma renal crisis, Incidence (epidemiology), Incidence, Hazard ratio, Acute Kidney Injury, Middle Aged, Europe, Treatment Outcome, Population Surveillance, Cohort, Hypertension, Female, 360 Social problems & social services, Proto-oncogene tyrosine-protein kinase Src, Research Article, Arterial hypertension, medicine.medical_specialty, 03 medical and health sciences, ENDOTHELIN-1, Internal medicine, Humans, Risk factor, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, SYSTEMIC-SCLEROSIS, Systemic, medicine.disease, Concomitant, lcsh:RC925-935, business
Abstract: Objectives To investigate the effect of ACE inhibitors (ACEi) on the incidence of scleroderma renal crisis (SRC) when given prior to SRC in the prospectively collected cohort from the European Scleroderma Trial and Research Group (EUSTAR). Methods SSc patients without prior SRC and at least one follow-up visit were included and analyzed regarding SRC, arterial hypertension, and medication focusing on antihypertensive medication and glucocorticoids (GC). Results Out of 14,524 patients in the database, we identified 7648 patients with at least one follow-up. In 27,450 person-years (py), 102 patients developed SRC representing an incidence of 3.72 (3.06–4.51) per 1000 py. In a multivariable time-to-event analysis adjusted for age, sex, disease severity, and onset, 88 of 6521 patients developed SRC. The use of ACEi displayed an increased risk for the development of SRC with a hazard ratio (HR) of 2.55 (95% confidence interval (CI) 1.65–3.95). Adjusting for arterial hypertension resulted in a HR of 2.04 (95%CI 1.29–3.24). There was no evidence for an interaction of ACEi and arterial hypertension (HR 0.83, 95%CI 0.32–2.13, p = 0.69). Calcium channel blockers (CCB), angiotensin receptor blockers (ARB), endothelin receptor antagonists, and GC—mostly in daily dosages below 15 mg of prednisolone—did not influence the hazard for SRC. Conclusions ACEi in SSc patients with concomitant arterial hypertension display an independent risk factor for the development of SRC but are still first choice in SRC treatment. ARBs might be a safe alternative, yet the overall safety of alternative antihypertensive drugs in SSc patients needs to be further studied.
Published: 2020

2. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author: Elhai, M, Boubaya, M, Distler, O, Smith, V, Matucci-Cerinic, M, Sancho, JJ, Truchetet, ME, Braun-Moscovici, Y, Iannone, F, Novikov, PI, Lescoat, A, Siegert, E, Castellvi, I, Airo, P, Vettori, S, Langhe, E, Hachulla, E, Erler, A, Ananieva, L, Krusche, M, Lopez-Longo, FJ, Distler, JHW, Hunzelmann, N, Hoffmann-Vold, AM, Riccieri, V, Hsu, VM, Pozzi, MR, Ancuta, C, Rosato, E, Mihai, C, Kuwana, M, Saketkoo, LA, Chizzolini, C, Hesselstrand, R, Ullman, S, Yavuz, S, Rednic, S, Caimmi, C, Bloch-Queyrat, C, Allanore, Y, Guiducci, S, Walker, UA, Kyburz, D, Lapadula, G, Maurer, B, Jordan, S, Dobrota, R, Becvar, R, Sierakowsky, S, Bielecka, OK, Sulli, A, Cutolo, M, Cuomo, G, Nicoara, I, Kahan, A, Vlachoyiannopoulos, PG, Montecucco, CM, Caporali, R, Stork, J, Inanc, M, Carreira, PE, Novak, S, Czirjak, L, Varju, C, Kucharz, EJ, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Cozzi, F, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Wu, C, Marjanovic, Z, Faivre, H, Hij, D, Dhamadi, R, Wollheim, F, Scheja, A, Wuttge, DM, Andreasson, K, Martinovic, D, Balbir-Gurman, A, Trotta, F, Lo Monaco, A, Pellerito, R, Mauriziano, O, Caramaschi, P, Morovic-Vergles, J, Black, C, Denton, C, Damjanov, N, Henes, J, Santamaria, VO, Heitmann, S, Krasowska, D, Matthias, Hasler, P, Burkhardt, H, Himsel, A, Bajocchi, G, Da Silva, JAP, Salvador, MJ, Stamenkovic, B, Stankovic, A, Selmi, CF, De Santis, M, Tikly, M, Denisov, LN, Herrick, A, Muller-Ladner, U, Frerix, M, Tarner, I, Scorza, R, Puppo, F, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szucs, G, Mendoza, AZ, de la Puente, C, Giraldo, WAS, Midtvedt, O, Reiseter, S, Garen, T, Launay, D, Valesini, G, Ionescu, RM, Groseanu, L, Opris, D, Cornateanu, RS, Ionitescu, R, Gherghe, AM, Soare, A, Gorga, M, Bojinca, M, Milicescu, M, Sunderkotter, C, Kuhn, A, Sandorfi, N, Schett, G, Beyer, C, Meroni, P, Ingegnoli, F, Mouthon, L, De Keyser, F, Melsens, K, Cantatore, FP, Corrado, A, Iversen, L, von Muhlen, CA, Bohn, JM, Lonzetti, LS, Eyerich, K, Hein, R, Knott, E, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Houssiau, FA, Krummel-Lorenz, B, Saar, P, Aringer, M, Gunther, C, Westhovens, R, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Uprus, M, Otsa, K, Granel, B, Muller, CD, Radominski, SC, Azevedo, VF, Jimenez, S, Busquets, J, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Popa, S, Zenone, T, Pileckyte, M, Mathieu, A, Vacca, A, Sampaio-Barros, PD, Yoshinari, NH, Marangoni, RG, Martin, P, Fuocco, L, Stebbings, S, Highton, J, Chapman, P, O'Donnell, J, Stamp, L, Doube, A, Solanki, K, Veale, D, O'Rourke, M, Loyo, E, Li, MT, Mohamed, WAAA, Amoroso, A, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, CM, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Chirieac, R, Furst, D, Villiger, P, Adler, S, van Laar, J, Kayser, C, Fathi, N, Hassanien, M, Lefebvre, PGD, Rubio, SR, Exposito, MV, Chatelus, E, Sibilia, J, Gottenberg, JE, Chifflot, H, Litinsky, I, Emery, P, Buch, M, Del Galdo, F, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Lasky, JA, Cosentino, V, Kerzberg, E, Montoya, F, Bianchi, W, Carneiro, S, Maretti, GB, Bianchi, DV, Limonta, M, Lupi, ALBE, Lupi, E, Rosner, I, Rozenbaum, M, Slobodin, G, Boulman, N, Rimar, D, Couto, M, Kahl, S, Chen, F, McCloskey, D, Malveaux, H, Spertini, F, Ribi, C, Buss, G, Martin, T, Guffroy, A, Poindron, V, Chotchaeva, F, Mukhin, NA, Moiseev, S, EUSTAR Network, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan José, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I, Lescoat, Alain, Siegert, Elise, Castellví, Ivan, Airó, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, López-Longo, F. J., Distler, Jörg H W, Hunzelmann, Nicola, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M, Pozzi, Maria R, Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, and Cuomo, Giovanna
Subjects: Male, Vital capacity, systemic sclerosis, Pulmonary Fibrosis, Vital Capacity, Scleroderma, lung fibrosis, rituximab, skin fibrosis, immune system diseases, DLCO, hemic and lymphatic diseases, Immunology and Allergy, Medicine, Prospective Studies, Registries, skin and connective tissue diseases, Prospective cohort study, Lung, skin fibrosi, Skin, ddc:616, integumentary system, Orvostudományok, Middle Aged, Respiratory Function Tests, lung fibrosis, rituximab, skin fibrosis, systemic sclerosis, Treatment Outcome, lung fibrosi, Antirheumatic Agents, Systemic sclerosis, Rituximab, Female, systemic sclerosi, medicine.drug, Adult, medicine.medical_specialty, Immunology, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, FEV1/FVC ratio, Rheumatology, Internal medicine, Humans, Adverse effect, Propensity Score, Aged, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Systemic, Skin fibrosis, business.industry, medicine.disease, Fibrosis, Lung fibrosis, business
Abstract: ObjectiveTo assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.MethodsWe performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab.Results254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], pConclusionRituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.
Published: 2019

3. What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Author: Proudman S. M., Huq M., Stevens W., Wilson M. E., Sahhar J., Baron M., Hudson M., Pope J., Allanore Y., Distler O., Kowal-Bielecka O., Matucci-Cerinic M., H. L. Low A., Teng G. G., Law W. G., Santosa A., Nikpour M., Hill C., Lester S., Nash P., Ngian G. -S., Proudman S., Rischmueller M., Roddy J., Strickland G., Thakkar V., Walker J., Zochling J., Markland J., Robinson D., Jones N., Khalidi N., Docherty P., Kaminska E., Masetto A., Sutton E., Mathieu J. -P., Ligier S., Grodzicky T., LeClercq S., Thorne C., Gyger G., Smith D., Fortin P. R., Larche M., Abu-Hakima M., Rodriguez-Reyna T. S., Cabral A. R., Fritzler M., Avouac J., Walker U. A., Guiducci S., Riemekasten G., Air P., Hachulla E., Valentini G., Carreira P. E., Cozzi F., Gurman A. B., Braun-Moscovici Y., Damjanov N., Ananieva L. P., Scorza R., Jimenez S., Busquets J., Li M., Muller-Ladner U., Maurer B., Tyndall A., Lapadula G., Iannone F., Becvar R., Sierakowsky S., Cutolo M., Sulli A., Cuomo G., Vettori S., Rednic S., Nicoara I., Vlachoyiannopoulos P., Montecucco C., Caporali R., Novak S., Czirjak L., Varju C., Chizzolini C., Kucharz E. J., Kotulska A., Kopec-Medrek M., Widuchowska M., Rozman B., Mallia C., Coleiro B., Gabrielli A., Farge D., Hij A., Hesselstrand R., Scheja A., Wollheim F., Martinovic D., Govoni M., Lo Monaco A., Hunzelmann N., Pellerito R., Bambara L. M., Caramaschi P., Black C., Denton C., Henes J., Santamaria V. O., Heitmann S., Krasowska D., Seidel M., Oleszowsky M., Burkhardt H., Himsel A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Starovoytova M. N., Engelhart M., Strauss G., Nielsen H., Damgaard K., Szucs G., Mendoza A. Z., de la Puente Buijdos C., Giraldo W. A. S., Midtvedt O., Garen T., Launay D., Valesini G., Riccieri V., Ionescu R. M., Opris D., Groseanu L., Wigley F. M., Mihai C. M., Cornateanu R. S., Ionitescu R., Gherghe A. M., Gorga M., Dobrota R., Bojinca M., Schett G., Distler J. H., Meroni P., Zeni S., Mouthon L., De Keyser F., Smith V., Cantatore F. P., Corrado A., Ullman S., Iversen L., Pozzi M. R., Eyerich K., Hein R., Knott E., Szechinski J., Wiland P., Szmyrka-Kaczmarek M., Sokolik R., Morgiel E., Krummel-Lorenz B., Saar P., Aringer M., Gunther C., Anic B., Baresic M., Mayer M., Radominski S. C., de Souza Muller C., Azevedo V. F., Agachi S., Groppa L., Chiaburu L., Russu E., Zenone T., Stebbings S., Highton J., Stamp L., Chapman P., O'Donnell J., Solanki K., Doube A., Veale D., O'Rourke M., Loyo E., Rosato E., Pisarri S., Tanaseanu C. -M., Popescu M., Dumitrascu A., Tiglea I., Chirieac R., Ancuta C., Furst D. E., Kafaja S., Garcia de la Pena Lefebvre P., Rubio S. R., Exposito M. V., Sibilia J., Chatelus E., Gottenberg J. E., Chifflot H., Litinsky I., Venalis A., Butrimiene I., Venalis P., Rugiene R., Karpec D., Kerzberg E., Montoya F., Cosentino V., Low A. H. L., Teng G., Chan G., Lim A. Y. N., Ng S. C., Proudman, S. M., Huq, M., Stevens, W., Wilson, M. E., Sahhar, J., Baron, M., Hudson, M., Pope, J., Allanore, Y., Distler, O., Kowal-Bielecka, O., Matucci-Cerinic, M., H. L. Low, A., Teng, G. G., Law, W. G., Santosa, A., Nikpour, M., Hill, C., Lester, S., Nash, P., Ngian, G. -S., Proudman, S., Rischmueller, M., Roddy, J., Strickland, G., Thakkar, V., Walker, J., Zochling, J., Markland, J., Robinson, D., Jones, N., Khalidi, N., Docherty, P., Kaminska, E., Masetto, A., Sutton, E., Mathieu, J. -P., Ligier, S., Grodzicky, T., Leclercq, S., Thorne, C., Gyger, G., Smith, D., Fortin, P. R., Larche, M., Abu-Hakima, M., Rodriguez-Reyna, T. S., Cabral, A. R., Fritzler, M., Avouac, J., Walker, U. A., Guiducci, S., Riemekasten, G., Air, P., Hachulla, E., Valentini, G., Carreira, P. E., Cozzi, F., Gurman, A. B., Braun-Moscovici, Y., Damjanov, N., Ananieva, L. P., Scorza, R., Jimenez, S., Busquets, J., Li, M., Muller-Ladner, U., Maurer, B., Tyndall, A., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Cutolo, M., Sulli, A., Cuomo, G., Vettori, S., Rednic, S., Nicoara, I., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Kucharz, E. J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Hij, A., Hesselstrand, R., Scheja, A., Wollheim, F., Martinovic, D., Govoni, M., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Denton, C., Henes, J., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., de la Puente Buijdos, C., Giraldo, W. A. S., Midtvedt, O., Garen, T., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, S., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Stebbings, S., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Rosato, E., Pisarri, S., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Garcia de la Pena Lefebvre, P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Low, A. H. L., Teng, G., Chan, G., Lim, A. Y. N., and Ng, S. C.
Subjects: 0301 basic medicine, medicine.medical_specialty, Pediatrics, Survival, Immunology, Disease, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, Immunology and Allergy, Medicine, Multicentre registrie, 030203 arthritis & rheumatology, Clinical features, Cohort study, Multicentre registries, Systemic sclerosis, business.industry, Interstitial lung disease, Autoantibody, Clinical features, medicine.disease, 030104 developmental biology, Clinical feature, Cohort, business, Cohort study, Rheumatism
Abstract: Introduction The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (pConclusions This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.
Published: 2017

4. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database

Author: Wirz, E. G., Jaeger, V. K., Allanore, Y., Riemekasten, G., Hachulla, E., Distler, O., Airo, P., Carreira, P. E., Tikly, M., Vettori, S., Gurman, A. B., Damjanov, N., Muller-Ladner, U., Distler, J., Li, M., Hausermann, P., Walker, U. A., Ananieva, L., Heitmann, S., Rednic, S., Jimenez, S., Riccieri, V., Szmyrka-Kaczmarek, M., Farge, D., Lapadula, G., Matucci-Cerinic, M., Guiducci, S., Hunzelmann, N., Rosa Pozzi, M., Mihai, C., Veale, D., Hesselstrand, R., Mariok, E., Smith, V., Kucharz, E. J., Czirjak, L., Martinovic, D., Solanki, K., Mihaela Ancuta, C., Sibilia, J., Paola, C., Hassanien, M., Kahl, S., Woods, A., Vanthuyne, M., Ruxandra, I., Radominski, S. C., Lo Monaco, A., Corrado, A., Koehm, M., Maurizio, M., Radim, B., Loyo, E., Uprus, M., Pellerito, R., Zenone, T., Gabrielli, A., Kowal-Bielecka, O., Rozman, B., Scorza, R., Ann Saketkoo, L., Midtvedt, O., von Muhlen, C. A., Henes, J., Branimir, A., Hasler, P., Yavuz, S., Villiger, P., Krummel-Lorenz, B., Posa, M., Engelhart, M., Denton, C., Krasowska, D., de la Pena Lefebvre, P. G., Cozzi, F., Mouthon, L., Rosato, E., Carlo, S., Alegre Sancho, J. J., Mallia, C., Limonta, M., Seidel, M., Foti, R., Stamp, L., Ullman, S., Stebbings, S., Ortiz Santamaria, V., Del Galdo, F., De Langhe, E., Mathieu, A., Sunderkotter, C., Eyerich, K., Stamenkovic, B., Novak, S., Sampaio-Barros, P. D., Kayser, C., Litinsky, I., Couto, M., University of Zurich, Walker, U A, Wirz, Eg, Jaeger, Vk, Allanore, Y, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, Pe, Tikly, M, Vettori, Serena, Balbir Gurman, A, Damjanov, N, Müller Ladner, U, Distler, J, Li, M, Häusermann, P, Walker, Ua, and Eustar, Coauthors
Subjects: Male, Genetics and Molecular Biology (all), Pathology, Longitudinal study, Time Factors, Databases, Factual, Epidemiology, systemic sclerosis, 2745 Rheumatology, Kaplan-Meier Estimate, Severity of Illness Index, Biochemistry, Scleroderma, Risk Factors, Medizinische Fakultät, Immunology and Allergy, Longitudinal Studies, Prospective Studies, 610 Medicine & health, integumentary system, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, 3. Good health, Autoantibodies, Systemic Sclerosis, Cohort, 2723 Immunology and Allergy, Female, Adult, medicine.medical_specialty, Immunology, General Biochemistry, Genetics and Molecular Biology, Sex Factors, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Skin Ulcer, medicine, Humans, ddc:610, Proportional Hazards Models, 2403 Immunology, Scleroderma, Systemic, business.industry, medicine.disease, Clinical trial, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Diffuse, business
Abstract: Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud9s phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.
Published: 2016

5. Effect of Macitentan on the Development of New Ischemic Digital Ulcers in Patients With Systemic Sclerosis

Author: Khanna, D, Denton, Cp, Merkel, Pa, Krieg, T, Le Brun FO, Marr, A, Papadakis, K, Pope, J, Matucci Cerinic, M, Furst, De, Zochling, J, Stevens, W, Proudman, S, Feenstra, J, Youssef, P, Soroka, N, Tyabut, T, Mikhailova, Ei, Rashkov, R, Batalov, A, Yablanski, K, Keystone, E, Jones, N, Dunne, J, Masetto, A, Calabresse, Rj, Cabezas, Pc, Silva, Mo, Sariego, Ia, Escalente, Wj, Anić, B, Kaliterna, Dm, Morović Vergles, J, Novak, S, Prus, V, Artuković, M, Soukup, T, Bečvař, R, Fojtík, Z, Mouthon, L, Kollert, F, Krieg, Tm, Riemekasten, G, Lahner, N, Fierlbeck, G, Ahmadi Simab, K, Diehm, C, Szücs, G, Kumánovics, G, Nagy, G, Pal, S, Veeravalli, Sc, Danda, D, Ferri, Clodoveo, Cerinic, Mm, Cozzi, F, Ferraccioli, G, Wiland, P, Rudnicak, L, Zwolak, R, Roszkiewicz, J, Oleynikov, V, Nikulenkova, N, Lesnyak, O, Kaydashev, I, Kurytar, O, Piura, O, Chopyak, V, Chatterjee, S, Hsu, V, Hummers, L, Martin, R, Domsic, R, Schiopu, E, Shanahan, J, Murphy, Ft, Kaine, J, Davis, W, Grau, R, Eimon, A, Catoggio, Lj, Laborde, Ha, Caeiro, F, Savio, Vg, Amitrano, Cb, Vanthuyne, M, Zeng, X, Zhang, X, Zhu, P, Velásquez Franco CJ, Choueka, Ps, Sanchez, Pj, Hermann, W, Sticherling, M, Steinbrink, K, Hein, R, Aschoff, R, Sfikakis, P, Settas, L, Fraser, A, Veale, D, Balbir Gurman, A, Lidar, M, Litinsky, I, Levy, Y, Carrillo Vazquez SM, Rodriguez Reyna, T, Medrano Ramirez, G, Morales Torres, J, Pacheco Tena CF, Sanchez Ortiz, A, Vonk, Mc, Stebbings, S, Solanki, K, Steele, R, Ng, Kp, Zubrzycka Sienkiewicz, A, Brzosko, M, Szepietowski, Jc, Hrycaj, P, da Silva IF, dos Santos Lda, C, Coelho, Pj, Rios, G, Chernykh, T, Grunina, E, Stanislav, M, Ally, M, Kalla, A, Birlik, Am, Kovalenko, V, Petrov, A, Shevchuk, S, Stanislavchuk, M, Anderson, M, Herrick, A, Belch, J, Chung, L, Csuka, Me, Frech, T, Goldberg, A, Kahaleh, B, Mayes, Md, Rothfield, N, Simms, Rw, Spiera, R, Steen, V, Varga, J, Sikes, D, Derk, Ct, Kohen, M. D., and UCL - (SLuc) Service de rhumatologie
Subjects: 0301 basic medicine, Male, Settore MED/16 - REUMATOLOGIA, systemic sclerosis, Peripheral edema, Administration, Oral, law.invention, Scleroderma, chemistry.chemical_compound, 0302 clinical medicine, Randomized controlled trial, law, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, Sulfonamides, Endothelin-1, Medicine (all), General Medicine, Middle Aged, Administration, Female, medicine.symptom, BIOMEDICINA I ZDRAVSTVO. Temeljne medicinske znanosti, Oral, medicine.medical_specialty, Double-Blind Method, Fingers, Humans, Outcome Assessment (Health Care), Pyrimidines, Scleroderma, Systemic, Skin Ulcer, Anemia, Macitentan, Digital Ulcers, Systemic Sclerosis, Placebo, 03 medical and health sciences, Internal medicine, medicine, Adverse effect, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Macitentan, 030203 arthritis & rheumatology, business.industry, BIOMEDICINE AND HEALTHCARE. Basic Medical Sciences, Systemic, Skin ulcer, medicine.disease, Surgery, Clinical trial, 030104 developmental biology, chemistry, Inflammatory diseases Radboud Institute for Health Sciences [Radboudumc 5], business
Abstract: Contains fulltext : 172407.pdf (Publisher’s version ) (Closed access) IMPORTANCE: Digital ulcers in patients with systemic sclerosis are associated with pain and poor quality of life. Endothelin-1 promotes vasculopathy in systemic sclerosis after macitentan, an endothelin-1 blocker. OBJECTIVE: To evaluate the efficacy of macitentan in reducing the number of new digital ulcers in patients with systemic sclerosis. DESIGN, SETTING, AND PARTICIPANTS: Two international, randomized, double-blind, placebo-controlled trials (DUAL-1, DUAL-2) were conducted between January 2012 and February 2014. Participants were patients with systemic sclerosis and active digital ulcers at baseline. Target enrollment for each study was 285 patients. INTERVENTIONS: Patients were randomized (1:1:1) to receive oral doses of 3 mg of macitentan, 10 mg of macitentan, or placebo once daily and stratified according to number of digital ulcers at baseline (3). MAIN OUTCOMES AND MEASURES: The primary outcome for each trial was the cumulative number of new digital ulcers from baseline to week 16. Treatment effect was expressed as the ratio between treatment groups. RESULTS: In DUAL-1, among 289 randomized patients (mean age 51.2 years; 85.8% women), 226 completed the study. The adjusted mean number of new digital ulcers per patient over 16 weeks was 0.94 in the 3-mg macitentan group (n = 95) and 1.08 in the 10-mg macitentan group (n = 97) compared with 0.85 in the placebo group (n = 97) (absolute difference, 0.09 [95% CI, -0.37 to 0.54] for 3 mg of macitentan vs placebo and 0.23 [-0.27 to 0.72] for 10 mg of macitentan vs placebo). Among 265 patients randomized in DUAL-2 (mean age 49.6 years; 81.9% women), 216 completed the study. In DUAL-2, the adjusted mean number of new digital ulcers was 1.44 in the 3-mg macitentan group (n = 88) and 1.46 in the 10-mg macitentan group (n = 88) compared with 1.21 in the placebo group (n = 89) (absolute difference, 0.23 [95% CI, -0.35 to 0.82] for 3 mg of macitentan vs placebo and 0.25 [95% CI, -0.34 to 0.84] for 10 mg of macitentan vs placebo). Adverse events more frequently associated with macitentan than with placebo were headache, peripheral edema, skin ulcer, anemia, upper respiratory tract infection, diarrhea, and nasopharyngitis. CONCLUSIONS AND RELEVANCE: Among patients with systemic sclerosis and active ischemic digital ulcers, treatment with macitentan did not reduce new digital ulcers over 16 weeks. These results do not support the use of macitentan for the treatment of digital ulcers in this patient population. TRIAL REGISTRATION: clinicaltrials.gov Identifiers: NCT01474109, NCT01474122.
Published: 2016

6. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database

Author: Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marques, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., VALENTINI, Gabriele, Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Valentini, Gabriele, Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marque, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., Chizzolini, Carlo, and Westhovens, Rene
Subjects: Male, Systemic disease, Databases, Factual, Cross-sectional study, Joint Diseases/etiology/pathology/physiopathology, Systemic inflammation, Joint involvement, Scleroderma, systemic sclrosis, systemic inflemmetion, joint involvement, Tendons, Systemic sclerosi, Scleroderma, Localized, 0302 clinical medicine, data base, Immunopathology, joint radiography, Immunology and Allergy, Joints/pathology, scleroderma, 030212 general & internal medicine, nuclear magnetic resonance imaging, Range of Motion, Articular, skin and connective tissue diseases, rheumatic disease, ddc:616, interstitial lung disease, Joint contracture, Clinical Trials as Topic, Synovitis, Inflammation/etiology/pathology/physiopathology, integumentary system, article, Tendons/pathology, Middle Aged, musculoskeletal system, cohort analysis, Connective tissue disease, priority journal, Joint, Synoviti, Systemic sclerosis, Female, medicine.symptom, Joint Diseases, Human, musculoskeletal diseases, Adult, medicine.medical_specialty, hand radiography, Immunology, Scleroderma, Localized/etiology/*pathology, Auto-immunity, transplantation and immunotherapy [N4i 4], 03 medical and health sciences, SYSTEMIC SCLEROSIS, JOINT INVOLVEMENT, SYNOVITIS, JOINT CONTRACTURE, TENDON FRICTION RUB, Tendon friction rub, Rheumatology, Internal medicine, medicine, Humans, Health care ethics [NCEBP 5], Tendon, Aged, 030203 arthritis & rheumatology, Cross-Sectional Studie, Inflammation, skin disease, Scleroderma, Systemic, business.industry, echography, medicine.disease, major clinical study, tenosynovitis, Synovitis/etiology/pathology, clinical feature, body regions, Cross-Sectional Studies, Evaluation of complex medical interventions [NCEBP 2], Scleroderma, Systemic/complications/pathology/physiopathology, Joints, disease duration, business, Joint Disease, disease activity
Abstract: Objective.To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc).Methods.This study was cross-sectional, based on data collected on patients included in the European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) registry. We queried this database to extract data regarding global evaluation of patients with SSc and the presence of any clinical articular involvement: synovitis (tender and swollen joints), tendon friction rubs (rubbing sensation detected as the tendon was moved), and joint contracture (stiffness of the joints that decreased their range of motion). Overall joint involvement was defined by the occurrence of synovitis and/or joint contracture and/or tendon friction rubs.Results.We recruited 7286 patients with SSc; their mean age was 56 ± 14 years, disease duration 10 ± 9 years, and 4210 (58%) had a limited cutaneous disease subset. Frequencies of synovitis, tendon friction rubs, and joint contractures were 16%, 11%, and 31%, respectively. Synovitis, tendon friction rubs, and joint contracture were more prevalent in patients with the diffuse cutaneous subset and were associated together and with severe vascular, muscular, renal, and interstitial lung involvement. Moreover, synovitis had the highest strength of association with elevated acute-phase reactants taken as the dependent variable.Conclusion.Our results highlight the striking level of articular involvement in SSc, as evaluated by systematic examination in a large cohort of patients with SSc. Our data also show that synovitis, joint contracture, and tendon friction rubs are associated with a more severe disease and with systemic inflammation.
Published: 2010

7. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author: Tyndall, A. J., Bannert, B., Vonk, M., Airo, P., Cozzi, F., Carreira, P. E., Bancel, D. F., Allanore, Y., Muller Ladner, U., Distler, O., Iannone, F., Pellerito, R., Pileckyte, M., Miniati, I., Ananieva, L., Gurman, A. B., Damjanov, N., Mueller, A., Valentini, G., Riemekasten, G., Tikly, M., Hummers, L., Henriques, M. J. S., Caramaschi, P., Scheja, A., Rozman, B., Ton, E., Kumanovics, G., Coleiro, B., Feierl, E., Szucs, G., Von Muhlen, C. A., Riccieri, Valeria, Novak, S., Chizzolini, C., Kotulska, A., Denton, C., Coelho, P. C., Kotter, I., Simsek, I., La Pena, D. E., Lefebvre, P. G. D. L. P., Hachulla, E., Seibold, J. R., Rednic, S., Stork, J., Morovic Vergles, J., Walker, U. A., Tyndall, Aj, Bannert, B, Vonk, M, Airò, P, Cozzi, F, Carreira, Pe, Bancel, Df, Allanore, Y, MÜLLER LADNER, U, Distler, O, Iannone, F, Pellerito, R, Pileckyte, M, Miniati, I, Ananieva, L, Gurman, Ab, Damjanov, N, Mueller, A, Valentini, Gabriele, Riemekasten, G, Tikly, M, Hummers, L, Henriques, Mj, Caramaschi, P, Scheja, A, Rozman, B, Ton, E, Kumánovics, G, Coleiro, B, Feierl, E, Szucs, G, VON MÜHLEN, Ca, Riccieri, V, Novak, S, Chizzolini, C, Kotulska, A, Denton, C, Coelho, Pc, Kötter, I, Simsek, I, DE LA PENA LEFEBVRE, Pg, Hachulla, E, Seibold, Jr, Rednic, S, Stork, J, MOROVIC VERGLES, J, and Walker, Ua
Subjects: Lung Diseases, systemic sclreosis, risk factors, Male, Lung Diseases/mortality, Comorbidity, 030204 cardiovascular system & hematology, 0302 clinical medicine, Neoplasms, Epidemiology, Pulmonary fibrosis, Immunology and Allergy, skin and connective tissue diseases, Cause of death, ddc:616, integumentary system, Interstitial lung disease, Sepsis/mortality, Middle Aged, Prognosis, 3. Good health, Pneumonia/mortality, Cohort, Female, Neoplasms/mortality, Gastrointestinal Hemorrhage, Scleroderma, Systemic/*mortality, Adult, medicine.medical_specialty, Heart Diseases, Immunology, Auto-immunity, transplantation and immunotherapy [N4i 4], General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Rheumatology, Sepsis, Internal medicine, medicine, Humans, Gastrointestinal Hemorrhage/mortality, Risk factor, Health care ethics [NCEBP 5], Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, Proportional hazards model, Heart Diseases/mortality, Pneumonia, medicine.disease, Surgery, Evaluation of complex medical interventions [NCEBP 2], Heart failure, business, Epidemiologic Methods
Abstract: Item does not contain fulltext OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc. 01 oktober 2010
Published: 2010

8. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author: Maurer, B., Graf, N., Michel, B. A., Muller Ladner, U., Czirjak, L., Denton, C. P., Tyndall, A., Metzig, C., Lanius, V., Khanna, D., Distler, O., Arner, I. H., Cerinic, M. M., Guiducci, S., Walker, U., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Bielecka, O. K., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Vettori, S., Riemekasten, G., Rednic, S., Nicoara, I., Kahan, A., Allanore, Y., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Carreira, P. E., Novak, S., Varju, C., Chizzolini, C., Kucharz, E. J., Kotulska, A., Kopec Medrek, M., Widuchowska, M., Cozzi, F., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Hij, A., Airo, P., Hesselstrand, R., Scheja, A., Wollheim, F., Martinovic, D., Gurman, A. B., Braun Moscovici, Y., Govoni, Marcello, LO MONACO, Andrea, Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Damjanov, N., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Ananieva, L. P., Scorza, R., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., Buijdos, C. d. l. P., Sifuentes Giraldo, W. A., Midtvedt, O., Garen, T., Hachulla, E., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, P., Mouthon, L., Keyser, F. D., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., Muller, C. d. S., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Pisarri, S., Tanaseanu, C. M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Lefebvre, P. G. d. l. P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Chizzolini, Carlo, Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, Carlomaurizio, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Kucharz, Eugene J, Kotulska, Anna, Kopec Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicola, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthia, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Giraldo, Walter A. Sifuente, Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Dougla, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina Mihaela, Popescu, Monica, and University of Zurich
Subjects: Genetics and Molecular Biology (all), Male, Time Factors, Databases, Factual, systemic sclerosis, 2745 Rheumatology, computer.software_genre, Biochemistry, Severity of Illness Index, Outcomes Research, Qualitative Research, Systemic Sclerosis, Adult, Cohort Studies, Creatine Kinase, Decision Support Techniques, Deglutition Disorders, Dyspnea, Female, Fibrosis, Humans, Logistic Models, Middle Aged, Multivariate Analysis, Scleroderma, Diffuse, Sex Factors, Skin, Synovitis, Disease Progression, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Immunology and Allergy, Medicine (all), Scleroderma, skin fibrosis, skin and connective tissue diseases, ddc:616, EUSTAR, Univariate analysis, Database, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Orvostudományok, Diffuse, Connective tissue disease, Cohort, 2723 Immunology and Allergy, Cohort study, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, NO, outcomes research, qualitative research, Databases, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, medicine, Factual, 2403 Immunology, business.industry, medicine.disease, business, computer
Abstract: ObjectivesTo identify predictive parameters for the progression of skin fibrosis within 1 year in patients with diffuse cutaneous SSc (dcSSc).MethodsAn observational study using the EUSTAR database was performed. Inclusion criteria were dcSSc, American College of Rheumatology (ACR) criteria fulfilled, modified Rodnan skin score (MRSS) ≥7 at baseline visit, valid data for MRSS at 2nd visit, and available follow-up of 12±2 months. Worsening of skin fibrosis was defined as increase in MRSS >5 points and ≥25% from baseline to 2nd visit. In the univariate analysis, patients with progressive fibrosis were compared with non-progressors, and predictive markers with pResultsA total of 637 dcSSc patients were eligible. Univariate analyses identified joint synovitis, short disease duration (≤15 months), short disease duration in females/patients without creatine kinase (CK) elevation, low baseline MRSS (≤22/51), and absence of oesophageal symptoms as potential predictors for progressive skin fibrosis. In the multivariate analysis, by employing combinations of the predictors, 17 models with varying prediction success were generated, allowing cohort enrichment from 9.7% progressive patients in the whole cohort to 44.4% in the optimised enrichment cohort. Using a second validation cohort of 188 dcSSc patients, short disease duration, low baseline MRSS and joint synovitis were confirmed as independent predictors of progressive skin fibrosis within 1 year resulting in a 4.5-fold increased prediction success rate.ConclusionsOur study provides novel, evidence-based criteria for the enrichment of dcSSc cohorts with patients who experience worsening of skin fibrosis which allows improved clinical trial design.
Published: 2014

9. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

Author: Walker U. A., Tyndall A., Czirják L, Denton C. ., Farge Bancel D., Kowal Bielecka O., Müller Ladner U., Bocelli Tyndall C., Matucci Cerinic M., Riemekasten G. ., Brückner C. ., Airó P. ., Scarsi M. ., Scorza R. ., Beretta L. ., Cozzi F. ., Tiso F., Vonk Mc M. C., Hoogen Van Den, Fhj F. H. J., Wigley Fm F. M., Hummers L. ., Nevskaya T. ., Ananieva L. ., Miniati I. ., Tartaglia N. ., Lomater C. ., Balbir Gurman A. ., Braun Moscovici Y. ., Bambara Lm L. M., Caramaschi P. ., Ruocco L. ., Krieg T. ., Hunzelmann N. ., Varjú C. ., Carriera Pe P. E., Joven B. ., Iannone F. ., Lapadula G. ., Kahan A. ., Allanore Y. ., Gabrielli A. ., Imperatore M. ., Scheja A. ., Wollheim F. ., Damjanov N. ., Ostojic P. ., Saar P. ., Tarner Ih I. H., Kötter I. ., Bombardieri S. ., Bazzichi L. ., Papa Del N. ., Comina Dp D. P., Monaco Lo A. ., Corte La R. ., Hachulla E. ., Launay D. ., Distler O. ., Ciurea A. ., Sierakowski S. ., Mitchell H. ., Silver Rm R. M., Krasowska D. ., Michalska Jakubus M. ., Tikly M. ., Aboo N. ., Worm M. ., Klaus P. ., Rovenský J. ., Lukáčová O. ., Rozman B. ., Sipek A. ., Clemente Coelho P. ., Shoenfeld Y. ., Langewitch P. ., José Da Silva Ap A. P., Salvador Mj M. J., Kuhn A. ., Erdmann G. ., Bečvář R. ., Friedl E. ., Graninger W. ., Riccieri V. ., Caporali R. ., Montecucco C. ., Vlachoyiannopoulos P. ., Distler M. ., Reich K. ., Majdan M. ., Wielosz E. ., Rednic S. ., Laar Van Jm J. M., Heitmann S. ., Bruckner A. ., Himsel A. ., Riemann J. ., Meyringer R. ., Müller A. ., Martinovic D. ., Radic M. ., Sticherling M. ., Szekanecz Z. ., Szücs G. ., Giacomelli R. ., Marrelli A. ., Stamenkovic B. ., Stankovic A. ., Aringer M. ., Smolen Js J. S., Kucharz Ej E. J., Kotulska At A. T., Jablonska S. ., Blasczik M. ., Jun J. B. J. B., Mallia C. ., Coleiro B. ., Santamaria Vo V. O., Hinrichs R. ., Nielsen H. ., Cossutta R. ., Ionescu R. ., Opris D. ., Steinbrink K. ., Grundt B. ., Bajocchi G. ., Jiří Š. ., Lefebvre Pgdlp P. G. D. L. P., Mendoza ZeaAc A. C., Ribi C. ., Chizzolini C. ., Wisłowska M. ., Novak S. ., Indiveri F. ., Jacobsen S. ., Frandsen Pb P. B., Gorska Iz I. Z., Gran Tore J. ., Midtvedt Ø. ., Ramos Fo F. O., Rajcevska Ld L. D., Bozinovski G. ., Schöffel D. ., Sunderkötter C. ., Böhm M. ., Morović Vergles J. ., Čulo M. I. M. I., Cutolo M. ., Sulli A. ., Derk Ct C. T., Jimenez Sa S. A., Siakka P. ., Søndergaard K. ., Stengaard Pedersen K. ., Cabane J. ., Tiev Kp K. P., Mihai C. ., Sfrent Cornateanu R. ., Jendro M. ., Tuvik P. ., Antivalle M. ., Randisi G. ., Seidel M. ., Clarenbach R. ., Simsek I. ., Dinc A. ., Inanc M. ., Capraru Ms M. S., Capraru D. ., Bañegil I. ., Richter J. ., Alhasani S. ., Földvari I. ., Pinto S. ., Brandão F. ., VALENTINI, Gabriele, Walker, U. A., Tyndall, A., Czirják, L, Denton, C. ., Farge Bancel, D., Kowal Bielecka, O., Müller Ladner, U., Bocelli Tyndall, C., Matucci Cerinic, M., Riemekasten, G. ., Brückner, C. ., Airó, P. ., Scarsi, M. ., Scorza, R. ., Beretta, L. ., Cozzi, F. ., Tiso, F., Vonk Mc, M. C., Hoogen Van, Den, Fhj, F. H. J., Wigley Fm, F. M., Hummers, L. ., Nevskaya, T. ., Ananieva, L. ., Miniati, I. ., Tartaglia, N. ., Lomater, C. ., Balbir Gurman, A. ., Braun Moscovici, Y. ., Bambara Lm, L. M., Caramaschi, P. ., Valentini, Gabriele, Ruocco, L. ., Krieg, T. ., Hunzelmann, N. ., Varjú, C. ., Carriera Pe, P. E., Joven, B. ., Iannone, F. ., Lapadula, G. ., Kahan, A. ., Allanore, Y. ., Gabrielli, A. ., Imperatore, M. ., Scheja, A. ., Wollheim, F. ., Damjanov, N. ., Ostojic, P. ., Saar, P. ., Tarner Ih, I. H., Kötter, I. ., Bombardieri, S. ., Bazzichi, L. ., Papa Del, N. ., Comina Dp, D. P., Monaco Lo, A. ., Corte La, R. ., Hachulla, E. ., Launay, D. ., Distler, O. ., Ciurea, A. ., Sierakowski, S. ., Mitchell, H. ., Silver Rm, R. M., Krasowska, D. ., Michalska Jakubus, M. ., Tikly, M. ., Aboo, N. ., Worm, M. ., Klaus, P. ., Rovenský, J. ., Lukáčová, O. ., Rozman, B. ., Sipek, A. ., Clemente Coelho, P. ., Shoenfeld, Y. ., Langewitch, P. ., José Da Silva Ap, A. P., Salvador Mj, M. J., Kuhn, A. ., Erdmann, G. ., Bečvář, R. ., Friedl, E. ., Graninger, W. ., Riccieri, V. ., Caporali, R. ., Montecucco, C. ., Vlachoyiannopoulos, P. ., Distler, M. ., Reich, K. ., Majdan, M. ., Wielosz, E. ., Rednic, S. ., Laar Van Jm, J. M., Heitmann, S. ., Bruckner, A. ., Himsel, A. ., Riemann, J. ., Meyringer, R. ., Müller, A. ., Martinovic, D. ., Radic, M. ., Sticherling, M. ., Szekanecz, Z. ., Szücs, G. ., Giacomelli, R. ., Marrelli, A. ., Stamenkovic, B. ., Stankovic, A. ., Aringer, M. ., Smolen Js, J. S., Kucharz Ej, E. J., Kotulska At, A. T., Jablonska, S. ., Blasczik, M. ., Jun, J. B. J. B., Mallia, C. ., Coleiro, B. ., Santamaria Vo, V. O., Hinrichs, R. ., Nielsen, H. ., Cossutta, R. ., Ionescu, R. ., Opris, D. ., Steinbrink, K. ., Grundt, B. ., Bajocchi, G. ., Jiří, Š. ., Lefebvre Pgdlp, P. G. D. L. P., Mendoza ZeaAc, A. C., Ribi, C. ., Chizzolini, C. ., Wisłowska, M. ., Novak, S. ., Indiveri, F. ., Jacobsen, S. ., Frandsen Pb, P. B., Gorska Iz, I. Z., Gran Tore, J. ., Midtvedt, Ø. ., Ramos Fo, F. O., Rajcevska Ld, L. D., Bozinovski, G. ., Schöffel, D. ., Sunderkötter, C. ., Böhm, M. ., Morović Vergles, J. ., Čulo, M. I. M. I., Cutolo, M. ., Sulli, A. ., Derk Ct, C. T., Jimenez Sa, S. A., Siakka, P. ., Søndergaard, K. ., Stengaard Pedersen, K. ., Cabane, J. ., Tiev Kp, K. P., Mihai, C. ., Sfrent Cornateanu, R. ., Jendro, M. ., Tuvik, P. ., Antivalle, M. ., Randisi, G. ., Seidel, M. ., Clarenbach, R. ., Simsek, I. ., Dinc, A. ., Inanc, M. ., Capraru Ms, M. S., Capraru, D. ., Bañegil, I. ., Richter, J. ., Alhasani, S. ., Földvari, I. ., Pinto, S. ., Brandão, F. ., Ribi, Camillo, and Chizzolini, Carlo
Subjects: Male, Systemic disease, Databases, Factual, Cross-sectional study, Scleroderma, Immunopathology, Immunology and Allergy, Age of Onset, skin and connective tissue diseases, ddc:616, integumentary system, Nuclear Proteins, Orvostudományok, Middle Aged, Connective tissue disease, Extended Report, Raynaud Disease/etiology/immunology, DNA Topoisomerases, Type I, Nuclear Proteins/immunology, Female, Adult, medicine.medical_specialty, Scleroderma, Diffuse/complications/immunology, Immunology, Klinikai orvostudományok, Scleroderma, Systemic/complications/immunology, Risk Assessment, General Biochemistry, Genetics and Molecular Biology, systemic sclerosis, EULAR, risk assessment, Age Distribution, Sex Factors, Rheumatology, Scleroderma, Limited, medicine, Humans, Risk factor, Aged, Autoantibodies, Scleroderma, Systemic, business.industry, Autoantibody, Raynaud Disease, medicine.disease, Dermatology, Cross-Sectional Studies, Scleroderma, Diffuse, Scleroderma, Limited/complications/immunology, Age of onset, business, Autoantibodies/blood
Abstract: Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and Methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the ACR diagnostic criteria in participating centres. We aimed to characterize demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were female. On multivariate analysis, scleroderma subsets (dcSSc vs. lcSSc), antibody status and age at onset of Raynaud’s phenomenon, but not gender were independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis appeared more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. The EUSTAR MEDS data base facilitates the analysis of clinical patterns in SSc and contributes to the standardised assessment and monitoring of SSc internationally.
Published: 2007

10. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients

Author: Ferri, C, Valentini, G, Cozzi, F, Sebastiani, M, Michelassi, C, La Montagna, G, Bullo, A, Cazzato, M, Tirri, E, Storino, F, Giuggioli, D, Cuomo, G, Rosada, M, Bombardieri, Stefano, Todesco, S, Tirri, G, Systemic Sclerosis Study Group of the Italian Society of Rheumatology, Ferri, C, Valentini, Gabriele, Cozzi, F, Sebastiani, M, Michelassi, C, LA MONTAGNA, G, Bullo, A, Cazzato, M, Tirri, E, Storino, F, Giuggioli, D, Cuomo, Giovanna, Rosada, M, Bombardieri, S, Todesco, S, Tirri, G, and SYSTEMIC SCLEROSIS STUDY GROUP OF THE ITALIAN SOCIETY OF, Rheumatology
Subjects: Male, Risk, medicine.medical_specialty, Multivariate analysis, MEDLINE, Systemic therapy, Skin Diseases, Scleroderma, Serology, Internal medicine, medicine, Humans, Sex Distribution, Survival rate, Systemic Sclerosis, Demographic, Clinical, and Serologic Features and Survival, Autoantibodies, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, General Medicine, Middle Aged, medicine.disease, Survival Rate, Multicenter study, Italy, Multivariate Analysis, Female, business, Follow-Up Studies
Published: 2002

11. VENTRICULAR ARRHYTHMIAS AND SUDDEN DEATH IN ATHLETES

Author: Bettini R, Guarnerio M, G. B. Durante, A. Del Favero, Marcello Disertori, Francesco Furlanello, Cozzi F, Gaetano Thiene, Giuseppe Inama, and Giuseppe Vergara
Subjects: Adult, Male, Risk, medicine.medical_specialty, Adolescent, Injury control, Arteriosclerosis, Accident prevention, Heart Ventricles, Physical Exertion, Poison control, Coronary Disease, Sudden death, General Biochemistry, Genetics and Molecular Biology, Death, Sudden, Electrocardiography, History and Philosophy of Science, Heart Conduction System, Internal medicine, medicine, Humans, Child, Mitral Valve Prolapse, biology, Athletes, business.industry, General Neuroscience, Arrhythmias, Cardiac, Cardiomyopathy, Hypertrophic, Middle Aged, biology.organism_classification, Surgery, Heart Block, Cardiology, Female, business, Sports
Published: 1984

12. CT for assessing feasibility of separation of thoracopagus twins

Author: Cozzi F, Rossi P, and Iannaccone G
Subjects: Radiography, Abdominal, Cardiac Catheterization, business.industry, Separation (statistics), Infant, Newborn, medicine.disease, Shunting, Contrast medium, medicine.anatomical_structure, Fetal Heart, Pregnancy, Conjoined twins, medicine, Humans, Radiology, Nuclear Medicine and imaging, Female, Tomography, Barium Sulfate, Nuclear medicine, business, Vein, Tomography, X-Ray Computed, Digestive System, Twins, Conjoined
Abstract: The value of the angio-computed tomography technique for evaluating communication between cardiac cavities of the two hearts in conjoined twins is reported. After injecting contrast medium into the peripheral vein of one of the twins, the increase in density values of the heart in the other twin suggested shunting between the two hearts.
Published: 1981

13. Induction of supraventricular tachyarrhythmia at rest and during exercise with transoesophageal atrial pacing in the electrophysiological evaluation of asymptomatic athletes with Wolff-Parkinson-White syndrome

Author: Giuseppe Vergara, Bettini R, Marcello Disertori, Giuseppe Inama, Francesco Furlanello, Cozzi F, and Guarnerio M
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, Hemodynamics, Asymptomatic, Electrocardiography, Internal medicine, Atrial Fibrillation, medicine, Tachycardia, Supraventricular, Humans, Heart Atria, Child, Atrial tachycardia, biology, Athletes, business.industry, Cardiac Pacing, Artificial, Cardiac arrhythmia, Atrial fibrillation, medicine.disease, biology.organism_classification, Ajmaline, Atrial Flutter, Cardiology, Exercise Test, Female, Wolff-Parkinson-White Syndrome, Supraventricular tachycardia, medicine.symptom, Cardiology and Cardiovascular Medicine, business, medicine.drug, Follow-Up Studies, Sports
Abstract: Even today there is controversy as regards the best approach to asymptomatic or slightly symptomatic athletes with the WPM syndrome as regards fitness for sports activity, especially in some countries where the doctor is responsible for certifying sports fitness. This study concerns 84 asymptomatic or slightly symptomatic athletes (66 males, 18 females, mean age 21.7 years, range 12–44 years) who underwent a stimulation protocol the end-point of which was the induction of atrial fibrillation (or, if not possible, atrial tachyarrhythmia) in the basal state and during bicycle stress test with transesophageal atrial pacing. The 81 athletes in whom the end-point was reached were divided into two groups: Group I includes the 32 athletes with the shortest R–R interval between pre-excited beats ≥ 240 ms in the basal state and /or ≥ 210 ms during bicycle ergometer test, Group II includes the other 49 patients. The evaluation during exercise was not carried out in four athletes because of serious haemodynamic compromise due to the arrhythmia induced in the basal state. Only 21/32 athletes would have been included in Group I if only evaluated in the basal state. In 30/81 athletes (37%), there was discrepancy between the result of stimulation and the result of the usual non-invasive evaluation (Holter monitoring, ergometric stress test, ajmaline test). On average, 40 min are required for the performance of the study protocol except when the induced arrhythmia lasts more than 5 min. The follow-up of the Group I athletes ranged from 10 to 36 months (mean 20.5 months); three athletes became symptomatic and in two of these the clinical arrhythmia was like the one induced. On the basis of our results, we conclude that the induction of atrial fibrillation under stress is useful in screening athletes with WPM syndrome; and, as it is simple to perform and economical (in time, equipment and staff), we regard this protocol as fundamental for the electrophysiological evaluation of athletes with Wolff–Parkinson–White syndrome.
Published: 1988

14. Impaired immunogenicity to COVID-19 vaccines in autoimmune systemic diseases. High prevalence of non-response in different patients' subgroups

Author: Domenico Olivo, E. Pigatto, Francesca La Gualana, Giorgio Amato, Ilaria Cavazzana, Rosario Foti, Antonio Tavoni, Raffaele Brittelli, Tommaso Ferrari, Francesco Masini, Marcella Visentini, Stefano Angelo Santini, Dilia Giuggioli, Franco Franceschini, Vincenzo Raimondo, Laura Gragnani, Giuseppa Pagano Mariano, Poupak Fallahi, Vincenzo Aiello, Lorenzo Puccetti, C. Naclerio, Riccardo Meliconi, Giovanna Cuomo, Amelia Spinella, Ylenia Dal Bosco, Alessandro Antonelli, Daniela Scorpiniti, M. Vadacca, Piero Ruscitti, Milvia Casato, Elisa Visalli, Florenzo Iannone, Maurizio Caminiti, Fabio Cacciapaglia, Francesco Ursini, Clodoveo Ferri, T. Urraro, Rodolfo Caminiti, Monica Monti, Massimo L'Andolina, Giovanni Rechichi, Anna Linda Zignego, Roberto Giacomelli, Giuseppe Varcasia, Roberta Pellegrini, Franco Cozzi, Pietro Gigliotti, Giusy Elia, Ferri, C., Ursini, F., Gragnani, L., Raimondo, V., Giuggioli, D., Foti, R., Caminiti, M., Olivo, D., Cuomo, G., Visentini, M., Cacciapaglia, F., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Varcasia, G., Cavazzana, I., L'Andolina, M., Ruscitti, P., Vadacca, M., Gigliotti, P., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Dal Bosco, Y., Amato, G., Masini, F., Pagano Mariano, G., Brittelli, R., Aiello, V., Caminiti, R., Scorpiniti, D., Rechichi, G., Ferrari, T., Monti, M., Elia, G., Franceschini, F., Meliconi, R., Casato, M., Iannone, F., Giacomelli, R., Fallahi, P., Santini, S. A., Zignego, A. L., Antonelli, A., Ferri C., Ursini F., Gragnani L., Raimondo V., Giuggioli D., Foti R., Caminiti M., Olivo D., Cuomo G., Visentini M., Cacciapaglia F., Pellegrini R., Pigatto E., Urraro T., Naclerio C., Tavoni A., Puccetti L., Varcasia G., Cavazzana I., L'Andolina M., Ruscitti P., Vadacca M., Gigliotti P., La Gualana F., Cozzi F., Spinella A., Visalli E., Dal Bosco Y., Amato G., Masini F., Pagano Mariano G., Brittelli R., Aiello V., Caminiti R., Scorpiniti D., Rechichi G., Ferrari T., Monti M., Elia G., Franceschini F., Meliconi R., Casato M., Iannone F., Giacomelli R., Fallahi P., Santini S.A., Zignego A.L., and Antonelli A.
Subjects: Male, History, Polymers and Plastics, Binding Antibody Units, BAU, Antibodies, Viral, Gastroenterology, Industrial and Manufacturing Engineering, Cryoglobulinemic vasculitis, CV, Scleroderma, Systemic sclerosi, Systemic lupu, Anti-citrullinated protein antibodies, ACPA, Systemic vasculitis, Lupus Erythematosus, Systemic, Immunology and Allergy, Medicine, Viral, Prospective Studies, Prospective cohort study, Neutralizing, education.field_of_study, Autoimmune systemic diseases, COVID-19 vaccine, Cryoglobulinemic vasculitis, Neutralizing antibodies, Rheumatoid arthritis, Systemic lupus, Systemic sclerosis, Immunogenicity, Vaccination, Middle Aged, Neutralizing antibody, NAb, Rheumatoid factor, RF, Italy, Female, Rituximab, Systemic sclerosis, SSc, Adverse events, AEs, 2019-nCoV Vaccine mRNA-1273, Human, medicine.drug, medicine.medical_specialty, Systemic lupus erythematosus, SLE, Immunology, Population, Autoimmune systemic disease, Autoimmune Disease, Article, Antibodies, Autoimmune Diseases, Internal medicine, Neutralizing antibodie, Humans, Business and International Management, Seroconversion, education, Settore BIO/10 - BIOCHIMICA, Vaccine Potency, Rheumatoid arthriti, BNT162 Vaccine, Scleroderma, Systemic, Lupus Erythematosus, Cryoglobulinemic vasculiti, SARS-CoV-2, business.industry, Systemic, Systemic Vasculiti, COVID-19, medicine.disease, Antibodies, Neutralizing, Autoimmune systemic diseases, ASD, Prospective Studie, World Health Organization, WHO, Rheumatoid arthritis, RA, Systemic Vasculitis, business
Abstract: Autoimmune systemic diseases (ASD) may show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed to evaluate the seroconversion after the vaccination cycle and at 6-12-month follow-up, as well the safety and efficacy of vaccines in preventing COVID-19. The study included 478 unselected ASD patients (mean age 59 ± 15 years), namely 101 rheumatoid arthritis (RA), 38 systemic lupus erythematosus (SLE), 265 systemic sclerosis (SSc), 61 cryoglobulinemic vasculitis (CV), and a miscellanea of 13 systemic vasculitis. The control group included 502 individuals from the general population (mean age 59 ± 14SD years). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was evaluated by measuring serum IgG-neutralizing antibody (NAb) (SARS-CoV-2 IgG II Quant antibody test kit; Abbott Laboratories, Chicago, IL) on samples obtained within 3 weeks after vaccination cycle. The short-term results of our prospective study revealed significantly lower NAb levels in ASD series compared to controls [286 (53–1203) vs 825 (451–1542) BAU/mL, p < 0.0001], as well as between single ASD subgroups and controls. More interestingly, higher percentage of non-responders to vaccine was recorded in ASD patients compared to controls [13.2% (63/478), vs 2.8% (14/502); p < 0.0001]. Increased prevalence of non-response to vaccine was also observed in different ASD subgroups, in patients with ASD-related interstitial lung disease (p = 0.009), and in those treated with glucocorticoids (p = 0.002), mycophenolate-mofetil (p < 0.0001), or rituximab (p < 0.0001). Comparable percentages of vaccine-related adverse effects were recorded among responder and non-responder ASD patients. Patients with weak/absent seroconversion, believed to be immune to SARS-CoV-2 infection, are at high risk to develop COVID-19. Early determination of serum NAb after vaccination cycle may allow to identify three main groups of ASD patients: responders, subjects with suboptimal response, non-responders. Patients with suboptimal response should be prioritized for a booster-dose of vaccine, while a different type of vaccine could be administered to non-responder individuals.
Published: 2022

15. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author: Rossella, De Angelis, Dilia, Giuggioli, Gianluigi, Bajocchi, Lorenzo, Dagna, Giovanni, Zanframundo, Rosario, Foti, Fabio, Cacciapaglia, Giovanna, Cuomo, Alarico, Ariani, Edoardo, Rosato, Serena, Guiducci, Francesco, Girelli, Valeria, Riccieri, Elisabetta, Zanatta, Silvia, Bosello, Ilaria, Cavazzana, Francesca, Ingegnoli, Maria De, Santis, Giuseppe, Murdaca, Giuseppina, Abignano, Nicoletta, Romeo, Alessandra, Della Rossa, Maurizio, Caminiti, Annamaria, Iuliano, Giovanni, Ciano, Lorenzo, Beretta, Gianluca, Bagnato, Ennio, Lubrano, Ilenia, De Andres, Alessandro, Giollo, Marta, Saracco, Cecilia, Agnes, Federica, Lumetti, Amelia, Spinella, Luca, Magnani, Corrado, Campochiaro, Giacomo, De Luca, Veronica, Codullo, Elisa, Visalli, Francesco, Masini, Antonietta, Gigante, Silvia, Bellando-Randone, Greta, Pellegrino, Erika, Pigatto, Francesca, Dall'Ara, Maria Grazia, Lazzaroni, Elena, Generali, Gianna, Mennillo, Simone, Barsotti, Giuseppa Pagano, Mariano, Francesca, Calabrese, Federica, Furini, Licia, Vultaggio, Simone, Parisi, Clara Lisa, Peroni, Anna Maria, Risa, Davide, Rozza, Anna, Zanetti, Greta, Carrara, Giampiero, Landolfi, Carlo Alberto, Scirè, Gerolamo, Bianchi, Enrico, Fusaro, Gian Domenico, Sebastiani, Marcello, Govoni, Salvatore, D'Angelo, Franco, Cozzi, Andrea, Doria, Florenzo, Iannone, Carlo, Salvarani, Marco, Matucci-Cerinic, Clodoveo, Ferri, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, de Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S., Cavazzana, I., Ingegnoli, F., de Santis, M., Murdaca, G., Abignano, G., Romeo, N., Rossa, A. D., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., de Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., de Luca, Giacomo., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Dall'Ara, F., Lazzaroni, M. G., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Risa, A. M., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Ferri, C., De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Dall'Ara, Francesca, Lazzaroni, Maria Grazia, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Pagano Mariano, Giuseppa, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Risa, Anna Maria, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, Matucci-Cerinic, Marco, and Ferri, Clodoveo
Subjects: Male, medicine.medical_specialty, Vital capacity, Settore MED/16 - REUMATOLOGIA, Cross-sectional study, Immunology, Left, Socio-culturale, scleroderma, sex, systemic sclerosis, Disease, Ventricular Function, Left, Scleroderma, Systemic sclerosi, Rheumatology, Internal medicine, Sicca syndrome, Sex, Systemic sclerosis, Cross-Sectional Studies, Female, Humans, Italy, Registries, Sex Characteristics, Stroke Volume, Scleroderma, Systemic, Sjogren's Syndrome, medicine, LS8_2, Immunology and Allergy, Ventricular Function, Honeycombing, skin and connective tissue diseases, Ejection fraction, integumentary system, business.industry, Systemic, medicine.disease, Cohort, business
Abstract: ObjectiveThere is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics.MethodsA multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared.ResultsThe overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs.ConclusionOur study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.
Published: 2022

16. COVID-19 and systemic sclerosis: clinicopathological implications from Italian nationwide survey study

Author: Clodoveo Ferri, Dilia Giuggioli, Vincenzo Raimondo, Lorenzo Dagna, Valeria Riccieri, Elisabetta Zanatta, Serena Guiducci, Antonio Tavoni, Rosario Foti, Giovanna Cuomo, Rossella De Angelis, Franco Cozzi, Giuseppe Murdaca, Ilaria Cavazzana, Nicoletta Romeo, Veronica Codullo, Francesca Ingegnoli, Roberta Pellegrini, Giuseppe Varcasia, Alessandra Della Rossa, Maria De Santis, Giuseppina Abignano, Michele Colaci, Maurizio Caminiti, Massimo L'Andolina, Ennio Lubrano, Amelia Spinella, Federica Lumetti, Giacomo De Luca, Silvia Bellando-Randone, Elisa Visalli, Silvia Bilia, Daiana Giannini, Francesco Masini, Greta Pellegrino, Erika Pigatto, Elena Generali, Francesca Dall'Ara, Giuseppa Pagano Mariano, Simone Barsotti, Giorgio Pettiti, Giovanni Zanframundo, Raffaele Brittelli, Vincenzo Aiello, Daniela Scorpiniti, Tommaso Ferrari, Rodolfo Caminiti, Corrado Campochiaro, Salvatore D'Angelo, Florenzo Iannone, Marco Matucci-Cerinic, Andrea Doria, Mario Miccoli, Poupak Fallahi, Alessandro Antonelli, Riccardo Cecchetti, Pietro Gigliotti, Domenico Olivo, Francesco Ursini, Veronica Brusi, Riccardo Meliconi, Raffaele Scarpa, Enrico Fusaro, Anna Linda Zignego, Sabrina Rosaria Paparo, Francesca Ragusa, Giusy Elia, Silvia Martina Ferrari, Ferri, C., Giuggioli, D., Raimondo, V., Dagna, L., Riccieri, V., Zanatta, E., Guiducci, S., Tavoni, A., Foti, R., Cuomo, G., De Angelis, R., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Ingegnoli, F., Pellegrini, R., Varcasia, G., Rossa, A. D., De Santis, M., Abignano, G., Colaci, M., Caminiti, M., L'Andolina, M., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Bellando-Randone, S., Visalli, E., Bilia, S., Giannini, D., Masini, F., Pellegrino, G., Pigatto, E., Generali, E., Dall'Ara, F., Mariano, G. P., Barsotti, S., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Scorpiniti, D., Ferrari, T., Caminiti, R., Campochiaro, C., D'Angelo, S., Iannone, F., Matucci-Cerinic, M., Doria, A., Miccoli, M., Fallahi, P., Antonelli, A., Della Rossa, A., Pagano Mariano, G., Cecchetti, R., Gigliotti, P., Olivo, D., Ursini, F., Brusi, V., Meliconi, R., Scarpa, R., Fusaro, E., Zignego, A. L., Paparo, S. R., Ragusa, F., Elia, G., Ferrari, S. M., Ferri C., Giuggioli D., Raimondo V., Dagna L., Riccieri V., Zanatta E., Guiducci S., Tavoni A., Foti R., Cuomo G., De Angelis R., Cozzi F., Murdaca G., Cavazzana I., Romeo N., Codullo V., Ingegnoli F., Pellegrini R., Varcasia G., Rossa A.D., De Santis M., Abignano G., Colaci M., Caminiti M., L'Andolina M., Lubrano E., Spinella A., Lumetti F., De Luca G., Bellando-Randone S., Visalli E., Bilia S., Giannini D., Masini F., Pellegrino G., Pigatto E., Generali E., Dall'Ara F., Mariano G.P., Barsotti S., Pettiti G., Zanframundo G., Brittelli R., Aiello V., Scorpiniti D., Ferrari T., Caminiti R., Campochiaro C., D'Angelo S., Iannone F., Matucci-Cerinic M., Doria A., Miccoli M., Fallahi P., Antonelli A., Della Rossa A., Pagano Mariano G., Cecchetti R., Gigliotti P., Olivo D., Ursini F., Brusi V., Meliconi R., Scarpa R., Fusaro E., Zignego A.L., Paparo S.R., Ragusa F., Elia G., Ferrari S.M., and De Luca, Giacomo.
Subjects: medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Immunology, MEDLINE, COVID-19, Nationwide survey, Rheumatology, Family medicine, Systemic sclerosis, Immunology and Allergy, Medicine, business
Abstract: None
Published: 2021

17. Serum anti-GM2 and anti-GalNAc-GD1a IgG antibodies are biomarkers for acute canine polyradiculoneuritis

Author: Dawn Gourlay, S. Rupp, Paul Freeman, M. R. Jackson, H. C. Schenk, Annette Wessmann, Akos Pakozdy, P. Pazzi, S. K. Halstead, Peter K. Smith, Hugh J. Willison, Ezio Bianchi, Nicolas Granger, Gualtiero Gandini, F. Tirrito, Thomas R Harcourt-Brown, Sofie Bhatti, F. Cozzi, Marco Menchetti, Rodrigo Gutierrez-Quintana, Jacques Penderis, M. Le Chevoir, V. Mortera-Balsa, Angie Rupp, A. Tauro, Mark Lowrie, Josep Brocal, Edward Ives, Andrea Tipold, Holger A. Volk, Maurizio Dondi, L. Martinez-Anton, I. Mateo, Mihai Musteata, M. Deutschland, Clare Rusbridge, Roberto José-López, Z. Whitehead, Halstead S.K., Gourlay D.S., Penderis J., Bianchi E., Dondi M., Wessmann A., Musteata M., Le Chevoir M., Martinez-Anton L., Bhatti S.F.M., Volk H., Mateo I., Tipold A., Ives E., Pakozdy A., Gutierrez-Quintana R., Brocal J., Whitehead Z., Granger N., Pazzi P., Harcourt-Brown T., Jose-Lopez R., Rupp S., Schenk H.C., Smith P., Gandini G., Menchetti M., Mortera-Balsa V., Rusbridge C., Tauro A., Cozzi F., Deutschland M., Tirrito F., Freeman P., Lowrie M., Jackson M.R., Willison H.J., and Rupp A.
Subjects: Male, medicine.medical_specialty, Polyradiculoneuropathy, G(M2) Ganglioside, Pilot Projects, Dog, canine, Polyradiculoneuritis, Gastroenterology, Immunoglobulin G, Canine polyradiculoneuritis, GalNAc-GD1a, Dogs, Internal medicine, medicine, Animals, Humans, Clinical significance, Dog Diseases, Small Animals, biology, business.industry, Odds ratio, Confidence interval, carbohydrates (lipids), Cohort, biology.protein, lipids (amino acids, peptides, and proteins), Female, Antibody, business, Biomarkers
Abstract: Objectives: \ud A previous single-country pilot study indicated serum anti-GM2 and anti-GA1 anti-glycolipid antibodies as potential biomarkers for acute canine polyradiculoneuritis. This study aims to validate these findings in a large geographically heterogenous cohort.\ud \ud Materials and Methods: \ud Sera from 175 dogs clinically diagnosed with acute canine polyradiculoneuritis, 112 dogs with other peripheral nerve, cranial nerve or neuromuscular disorders and 226 neurologically normal dogs were screened for anti-glycolipid antibodies against 11 common glycolipid targets to determine the immunoglobulin G anti-glycolipid antibodies with the highest combined sensitivity and specificity for acute canine polyradiculoneuritis.\ud \ud Results: \ud Anti-GM2 anti-glycolipid antibodies reached the highest combined sensitivity and specificity (sensitivity: 65.1%, 95% confidence interval 57.6 to 72.2%; specificity: 90.2%, 95% confidence interval 83.1 to 95.0%), followed by anti-GalNAc-GD1a anti-glycolipid antibodies (sensitivity: 61.7%, 95% confidence interval 54.1 to 68.9%; specificity: 89.3%, 95% confidence interval 82.0 to 94.3%) and these anti-glycolipid antibodies were frequently present concomitantly. Anti-GA1 anti-glycolipid antibodies were detected in both acute canine polyradiculoneuritis and control animals. Both for anti-GM2 and anti-GalNAc-GD1a anti-glycolipid antibodies, sex was found a significantly associated factor with a female to male odds ratio of 2.55 (P=0.0096) and 3.00 (P=0.0198), respectively. Anti-GalNAc-GD1a anti-glycolipid antibodies were more commonly observed in dogs unable to walk (odds ratio 4.56; P=0.0076).\ud \ud Clinical Significance: \ud Anti-GM2 and anti-GalNAc-GD1a immunoglobulin G anti-glycolipid antibodies represent serum biomarkers for acute canine polyradiculoneuritis.
Published: 2021

18. Comparison of intranasal versus intravenous midazolam for management of status epilepticus in dogs: A multi‐center randomized parallel group clinical study

Author: Joan R. Coates, Enrice Huenerfauth, Theresa E. Pancotto, Rodolfo Cappello, Johannes Erath, Marios Charalambous, John H. Rossmeisl, Bart J. G. Broeckx, An Vanhaesebrouck, Simon R. Platt, Luisa De Risio, Gualtiero Gandini, Mihai Musteata, Daisuke Hasegawa, Andrea Tipold, Sofie Bhatti, Holger A. Volk, Antonella Gallucci, Laura Porcarelli, Francesca Cozzi, Luc Van Ham, Daniele Corlazzoli, Federica Tirrito, Charalambous M., Volk H.A., Tipold A., Erath J., Huenerfauth E., Gallucci A., Gandini G., Hasegawa D., Pancotto T., Rossmeisl J.H., Platt S., De Risio L., Coates J.R., Musteata M., Tirrito F., Cozzi F., Porcarelli L., Corlazzoli D., Cappello R., Vanhaesebrouck A., Broeckx B.J.G., Van Ham L., Bhatti S.F.M., Charalambous, Marios [0000-0002-6964-6307], Hasegawa, Daisuke [0000-0002-7554-9108], Broeckx, Bart JG [0000-0001-6742-3911], and Apollo - University of Cambridge Repository
Subjects: Male, PHARMACOKINETICS, Midazolam, Sedation, canine, nasal, CHILDREN, Standard Article, Status epilepticus, Epilepsy, Dogs, Status Epilepticus, Statistical significance, medicine, Animals, ACUTE SEIZURES, Ketamine, Dog Diseases, Veterinary Sciences, benzodiazepines, Adverse effect, Administration, Intranasal, lcsh:Veterinary medicine, PEDIATRIC-PATIENTS, General Veterinary, emergency, BLOOD-BRAIN-BARRIER, business.industry, RECTAL DIAZEPAM, KETAMINE, medicine.disease, Standard Articles, TRANSPORT, Clinical trial, NASAL DRUG-DELIVERY, Neurology, Anesthesia, Injections, Intravenous, lcsh:SF600-1100, epilepsy, Female, SMALL ANIMAL, benzodiazepine, medicine.symptom, business, medicine.drug
Abstract: Background: The intranasal (IN) route for rapid drug administration in patients with brain disorders, including status epilepticus, has been investigated. Status epilepticus is an emergency, and the IN route offers a valuable alternative to other routes, especially when these fail. Objectives: To compare IN versus IV midazolam (MDZ) at the same dosage (0.2 mg/kg) for controlling status epilepticus in dogs. Animals Client-owned dogs (n = 44) with idiopathic epilepsy, structural epilepsy, or epilepsy of unknown origin manifesting as status epilepticus. Methods: Randomized parallel group clinical trial. Patients were randomly allocated to the IN-MDZ (n = 21) or IV-MDZ (n = 23) group. Number of successfully treated cases (defined as seizure cessation within 5 minutes and lasting for >= 10 minutes), seizure cessation time, and adverse effects were recorded. Comparisons were performed using the Fisher's exact and Wilcoxon rank sum tests with statistical significance set at alpha < .05. Results: IN-MDZ and IV-MDZ successfully stopped status epilepticus in 76% and 61% of cases, respectively (P = .34). The median seizure cessation time was 33 and 64 seconds for IN-MDZ and IV-MDZ, respectively (P = .63). When the time to place an IV catheter was taken into account, IN-MDZ (100 seconds) was superior (P = .04) to IV-MDZ (270 seconds). Sedation and ataxia were seen in 88% and 79% of the dogs treated with IN-MDZ and IV-MDZ, respectively. Conclusions and Clinical Importance: Both routes are quick, safe, and effective for controlling status epilepticus. However, the IN route demonstrated superiority when the time needed to place an IV catheter was taken into account.
Published: 2019

19. Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments

Author: M. L. Aprile, Rosario Foti, Ruscitti Piero, Giuseppe Varcasia, Ilaria Cavazzana, Nicoletta Romeo, Riccardo Meliconi, Serena Lorini, Serena Guiducci, Amelia Spinella, Roberto Giacomelli, Vincenzo Aiello, Alessandra Della Rossa, Giorgio Amato, Daiana Giannini, Vincenzo Raimondo, Francesco Caso, Ennio Lubrano, Silvia Bosello, Maurizio Caminiti, Monica Monti, Tasso Marco, Francesca Ingegnoli, Giorgio Pettiti, Massimo L'Andolina, Salvatore D'Angelo, Francesca Ragusa, Elisabetta Zanatta, Giacomo De Luca, Riccardo Cecchetti, Franco Franceschini, Greta Pellegrino, Silvia Bellando-Randone, Silvia Martina Ferrari, Micaela Fredi, Veronica Brusi, Lorenzo Dagna, Giusy Elia, Fabio Cacciapaglia, Francesco Ursini, Giuseppina Abignano, Sebastiano Lorusso, Clodoveo Ferri, Anna Linda Zignego, Rodolfo Caminiti, Sabrina Rosaria Paparo, Raffaele Brittelli, Liala Moschetti, Elena Generali, Marco Matucci-Cerinic, Roberta Pellegrini, Ylenia Dal Bosco, Giovanni Zanframundo, Domenico Olivo, Tommaso Ferrari, Alessandro Antonelli, M. Vadacca, Andrea Doria, Pietro Gigliotti, Poupak Varcasia, Enrico Fusaro, Elisa Visalli, Simone Barsotti, Giuseppa Pagano Mariano, Giovanna Cuomo, Florenzo Iannone, Maria De Santis, Valeria Mazzi, Giuseppe Murdaca, Michele Colaci, Silvia Bilia, Franco Cozzi, Dilia Giuggioli, Corrado Campochiaro, Valeria Riccieri, Erika Pigatto, Laura Gragnani, Ilenia Di Cola, Daniela Scorpiniti, Mario Miccoli, Francesco Masini, Veronica Codullo, Rossella De Angelis, Federica Lumetti, Antonio Tavoni, Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L’Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Piero, Ruscitti, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Rossa, Alessandra Della, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Dal Bosco, Ylenia, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Gragnani, Laura, Monti, Monica, Lorini, Serena, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Barsotti, Simone, Aprile, Maria Letizia, Marco, Tasso, Miccoli, Mario, Bosello, Silvia, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Varcasia, Poupak, Antonelli, Alessandro, L'Andolina, Massimo, Della Rossa, Alessandra, Pagano Mariano, Giuseppa, Rosaria Paparo, Sabrina, Martina Ferrari, Silvia, Letizia Aprile, Maria, Linda Zignego, Anna, Ferri C., Giuggioli D., Raimondo V., L'andolina M., Dagna L., Tavoni A., Caso F., Ursini F., Ruscitti P., Caminiti M., Foti R., Riccieri V., Guiducci S., Pellegrini R., Zanatta E., Varcasia G., Olivo D., Gigliotti P., Cuomo G., Murdaca G., Cecchetti R., De Angelis R., Romeo N., Ingegnoli F., Cozzi F., Codullo V., Cavazzana I., Colaci M., Abignano G., De Santis M., Lubrano E., Fusaro E., Della Rossa A., Spinella A., Lumetti F., De Luca G., Bellando-Randone S., Visalli E., Dal Bosco Y., Amato G., Giannini D., Bilia S., Masini F., Pellegrino G., Pigatto E., Generali E., Mariano G.P., Pettiti G., Zanframundo G., Brittelli R., Aiello V., Caminiti R., Scorpiniti D., Ferrari T., Campochiaro C., Brusi V., Fredi M., Moschetti L., Cacciapaglia F., Gragnani L., Monti M., Lorini S., Paparo S.R., Ragusa F., Mazzi V., Elia G., Ferrari S.M., Di Cola I., Vadacca M., Lorusso S., Barsotti S., Aprile M.L., Marco T., Miccoli M., Bosello S., Matucci-Cerinic M., D'angelo S., Doria A., Franceschini F., Meliconi R., Iannone F., Giacomelli R., Zignego A.L., Fallahi P., and Antonelli A.
Subjects: medicine.medical_specialty, Settore MED/16 - REUMATOLOGIA, Referral, Coronavirus disease 2019 (COVID-19), autoimmune systemic disease, systemic sclerosis, Population, COVID-19, SARS-CoV-2, arthritis, autoimmune systemic diseases, connective tissue diseases, interstitial lung disease, rheumatic diseases, Arthritis, Keywords: COVID-19, Scleroderma, Autoimmune Diseases, Covid-19, Autoimmune systemic diseases, Connective tissue diseases, Interstitial lung disease, Rheumatic diseases, Systemic sclerosis, Internal medicine, Rheumatic Diseases, Drug Discovery, Pandemic, medicine, Humans, education, rheumatic disease, Lung, Pandemics, Pharmacology, education.field_of_study, Aspirin, business.industry, covid 19, medicine.disease, arthriti, connective tissue disease, business, medicine.drug
Abstract: Background: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations. Objective: This study aims to investigate the prevalence of symptomatic Covid-19 and its correlations with both organ involvement and ongoing treatments in a large series of Italian ASD patients during the first wave of pandemic. Methods: Our multicenter telephone 6-week survey included 3,029 unselected ASD patients enrolled at 36 tertiary referral centers of northern, central, and southern Italian macro-areas with different diffusion of the pandemic. Symptomatic SARS-CoV-2 infection was classified as definite Covid-19 (presence of symptoms plus positive oral/nasopharyngeal swabs) or highly suspected Covid-19 (highly suggestive symptoms, in the absence of a swab testing). Results: A significantly higher prevalence of definite plus highly suspected Covid-19 compared to the Italian general population was detected in the whole ASD series (p=.000), as well as in patients from the three macro-areas (p=.000 in all). Statistically higher prevalence of Covid-19 was also found in connective tissue diseases compared to chronic arthritis subgroup (p=.000) and in ASD patients with pre-existing interstitial lung involvement (p=.000). Patients treated with either conventional disease-modifying anti-rheumatic drugs (DMARDs) and/or biological DMARDs showed a significantly lower prevalence of Covid-19 (p=.000 in both). Finally, scleroderma patients undergoing low-dose aspirin showed a significantly lower rate of Covid-19 compared to those without (p=0.003). Conclusion: The higher prevalence of Covid-19 in ASD patients, along with the significant correlations with important clinical features and therapeutic regimens, suggests the need to develop targeted prevention/management strategies during the current pandemic wave.
Published: 2021

20. Agreement of surgeon's perception of the effectiveness of spinal cord decompression with findings on postoperative magnetic resonance imaging for dogs surgically treated for intervertebral disk extrusion

Author: Martina Bonaldi, Sara Conti, Rocco Lombardo, Francesca Cozzi, Federica Tirrito, Tirrito, F, Cozzi, F, Bonaldi, M, Conti, S, and Lombardo, R
Subjects: musculoskeletal diseases, medicine.medical_specialty, genetic structures, 040301 veterinary sciences, Postoperative mri, 0403 veterinary science, 03 medical and health sciences, Dogs, medicine, Animals, Humans, Dog Diseases, Intervertebral Disc, 030304 developmental biology, Retrospective Studies, Surgeons, 0303 health sciences, General Veterinary, medicine.diagnostic_test, business.industry, Magnetic resonance imaging, 04 agricultural and veterinary sciences, compression, degeneration, association, small-breed dogs, medicine.disease, Magnetic Resonance Imaging, Surgery, Intervertebral disk, Spinal decompression, business, Spinal Cord Compression, Intervertebral Disc Displacement
Abstract: OBJECTIVE To determine the accuracy of the surgeon's perception versus postoperative MRI findings in assessing the effectiveness of spinal cord decompression achieved in dogs surgically treated for intervertebral disk extrusion (IVDE) and whether postoperative MRI findings were more likely to be associated with various outcomes. ANIMALS 68 dogs surgically treated for cervical or thoracolumbar IVDE. PROCEDURES Data on clinical, neurologic, pre- and postoperative MRI, and intraoperative findings as well as outcomes and recovery times (6-month follow-up period) were prospectively collected and compared between various groups. RESULTS 54 (79%) dogs had thoracolumbar IVDE, and 14 (21%) had cervical IVDE. Median degree of spinal cord compression as assessed on transverse T2-weighted MRI images was 45.6% before surgery and 8.8% after surgery. The correlation between surgeons' perception (n = 3) and postoperative MRI findings for the degree of spinal cord decompression achieved was only fair (κ = 0.40). Unsatisfactory spinal cord decompression as assessed via postoperative MRI was associated with severity of preoperative neurologic grade and preoperative compression, thoracolumbar (vs cervical) IVDE, and ventral (vs ventrolateral or dorsolateral) circumferential distribution of extruded material. Satisfactory (vs unsatisfactory) decompression as assessed via MRI was associated with a lower postoperative neurologic grade, greater likelihood of a successful outcome, and lower mean recovery time. CONCLUSIONS AND CLINICAL RELEVANCE Results suggested that for dogs surgically treated for IVDE, the surgeon's perception of adequate spinal cord decompression may be less reliable than postoperative MRI findings. Postoperative MRI appeared particularly useful for dogs with a severe preoperative neurologic status, severe preoperative spinal cord compression, and thoracolumbar IVDE.
Published: 2020

21. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

Author: Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, W., Jordan, S., Graf, N., de Oliveira Pena, J., Curram, J., Allanore, Y., Matucci-Cerinic, M., Pope, J. E., Denton, C. P., Khanna, D., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., Sarzi Puttini, P., Ege Üniversitesi, Chizzolini, Carlo, Allali, Danièle, University of Zurich, and Distler, Oliver
Subjects: INVOLVEMENT, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, Time Factors, Databases, Factual, Skin Diseases/etiology/mortality/physiopathology, PREDICTION, Fibrosi, 2745 Rheumatology, Diffuse/complications/mortality/pathology, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, Kaplan-Meier Estimate, ddc:616.07, Severity of Illness Index, Scleroderma, Cohort Studies, PROGNOSTIC-FACTORS, Fibrosis, Medicine and Health Sciences, scleroderma, Lung, Skin, integumentary system, progressive skin fibrosis, Lung function decline, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, DEATH, Middle Aged, ddc, Europe, VARIABILITY, factual, Cohort, Visceral organ progression, 2723 Immunology and Allergy, Disease Progression, Female, Survival Analysi, Life Sciences & Biomedicine, Cohort study, Human, Adult, Skin/pathology, medicine.medical_specialty, databases, All-cause death, risk analysis, diffuse cutaneous systemic sclerosis, 610 Medicine & health, IMPROVEMENT, Systemic Sclerosis, Skin Diseases, THICKNESS SCORE, VALIDATION, Databases, FEV1/FVC ratio, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Humans, Factual, Survival analysis, 2403 Immunology, Science & Technology, Proportional hazards model, business.industry, Surrogate endpoint, MORTALITY, Skin Disease, fibrosis, Progressive skin fibrosi, Lung/physiopathology, biomarkers, Diffuse cutaneous systemic sclerosi, medicine.disease, Survival Analysis, all-cause death, lung function decline, visceral organ progression, adult, cohort studies, databases, factual, disease progression, female, humans, Scleroderma, Diffuse, Cohort Studie, business
Abstract: PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.
Published: 2019

22. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis

Author: Becker, Mike, Graf, Nicole, Sauter, Rafael, Allanore, Yannick, Curram, John, Denton, Christopher P., Khanna, Dinesh, Matucci-Cerinic, Marco, Pena, Janethe de Oliveira, Pope, Janet E., Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Ege Üniversitesi, University of Zurich, Distler, Oliver, Chizzolini, Carlo, Allai, Daniela, Becker, M., Graf, N., Sauter, R., Allanore, Y., Curram, J., Denton, C. P., Khanna, D., Matucci-Cerinic, M., de Oliveira Pena, J., Pope, J. E., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., and Sarzi Puttini, P.
Subjects: INVOLVEMENT, SELECTION, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, predictive factors, systemic sclerosis, 2745 Rheumatology, epidemiologic methods, morbidity, Disease, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, INTERSTITIAL LUNG-DISEASE, disease worsening, mortality, predictive factors, systemic sclerosis, predictive factor, disease worsening, DESIGN, middle aged, Medicine and Health Sciences, FIBROSIS, scleroderma, SKIN THICKNESS SCORE, mortality, skin and connective tissue diseases, Prospective cohort study, humans, lung diseases, ddc:616, education.field_of_study, heart diseases, integumentary system, clinical trials as topic, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, Interstitial lung disease, follow-up studies, ddc, female, Cohort, 2723 Immunology and Allergy, europe, Life Sciences & Biomedicine, CLINICAL-TRIALS, survival rate, medicine.medical_specialty, Population, 610 Medicine & health, disease progression, male, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, REGRESSION, medicine, severity of illness index, education, Survival rate, METAANALYSIS, 2403 Immunology, Science & Technology, Scleroderma, Systemic, business.industry, MORTALITY, systemic, medicine.disease, prospective studies, Clinical trial, prognosis, scleroderma, diffuse, scleroderma, systemic, Scleroderma, Diffuse, business
Abstract: PubMed: 31227488, Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.
Published: 2019

23. Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients

Author: E. Pigatto, Diletta Peluso, P. Miatton, Franco Cozzi, Elisabetta Zanatta, Luigi P. Badano, Leonardo Punzi, K. Bourji, Pamela Polito, Pigatto, E, Peluso, D, Zanatta, E, Polito, P, Miatton, P, Bourji, K, Badano, L, Punzi, L, and Cozzi, F
Subjects: Adult, Male, 3d-echocardiography, Right ventricular function, Systemic sclerosis, Rheumatology, medicine.medical_specialty, lcsh:Internal medicine, Heart disease, Ventricular Dysfunction, Right, Echocardiography, Three-Dimensional, lcsh:Medicine, Logistic regression, Sensitivity and Specificity, Scleroderma, Systemic sclerosi, Predictive Value of Tests, Internal medicine, Medicine, Humans, skin and connective tissue diseases, lcsh:RC31-1245, Pathological, Aged, Pressure overload, Scleroderma, Systemic, integumentary system, business.industry, lcsh:R, Reproducibility of Results, Right ventricular function, 3d-echocardiography, Systemic sclerosis, Middle Aged, medicine.disease, medicine.anatomical_structure, Italy, Ventricle, Echocardiography, Case-Control Studies, Cardiology, Vascular resistance, Female, business
Abstract: The impairment of the right ventricle (RV) in systemic sclerosis (SSc) is usually related to pulmonary arterial hypertension (PAH). New echocardiographic techniques, such as 3-dimensional echocardiography (3DE) and 2-dimensional speckle tracking (2DSTE), allow an accurate evaluation of the RV function. The aim of this study was to evaluate the RV function using 3DE and 2DSTE in SSc patients with no history of heart disease and no PAH. Forty-five SSc patients, 42 females and 3 males, 28 with limited cutaneous SSc (lcSSc) and 17 with diffuse cutaneous SSc (dcSSc), were studied. Forty-three age- and gender-matched healthy subjects were enrolled as controls. All of them underwent a 3DE and 2DSTE ecocardiographic evaluation of the RV function. Systolic pulmonary arterial pressure (sPAP) and total pulmonary vascular resistance (tPVR) were also estimated by power doppler. RV echocardiographic parameters were compared in the different subsets of SSc patients. A statistical analysis was performed by t-test, ANOVA and multiple logistic regression. RV areas in 2DSTE and volumes in 3DE were higher and RV function parameters were reduced in SSc patients compared with controls. Also sPAP and tVPR were higher, but they did not reach pathological values. Echocardiographic alterations were more pronounced in patients with lcSSc. 3DE and 2DSTE echocardiography allowed us to detect morphological and functional alterations of the RV in a group of SSc patients with no clinical signs of heart disease and no PAH. These patients had significantly higher sPAP and tPVR than healthy controls without reporting values compatible with PAH. These data suggest that RV alterations are related to a pressure overload rather than to an intrinsic myocardial involvement in SSc.
Published: 2015

24. 'To Be or Not To Be,' Ten Years After: Evidence for Mixed Connective Tissue Disease as a Distinct Entity

Author: Simona Rednic, L. P. Ananyeva, Olga Koneva, Katarina Simic Pasalic, Marta Mosca, László Czirják, Valeria Riccieri, Guido Valesini, Stefano Bombardieri, Susanna Cappelli, Csaba György Kiss, Maria Magdalena Tamas, Ruxandra Ionescu, Mike O Becker, Marco Matucci Cerinic, S. Cardarelli, Gabriela Riemekasten, Franco Cozzi, Nemanja Damjanov, Yannick Allanore, Rosaria Talarico, Gabriele Valentini, Silvia Bellando Randone, Giovanna Cuomo, Martin Aringer, Mislav Radić, Anne Kathrin Tausche, Alberto Sulli, Serena Guiducci, Dušanka Martinović, Maurizio Cutolo, Daniela Opris, Cappelli, S, Bellando Randone, S, Martinović, D, Tamas, Mm, Pasalić, K, Allanore, Y, Mosca, M, Talarico, R, Opris, D, Kiss, Cg, Tausche, Ak, Cardarelli, S, Riccieri, V, Koneva, O, Cuomo, Giovanna, Becker, Mo, Sulli, A, Guiducci, S, Radić, M, Bombardieri, S, Aringer, M, Cozzi, F, Valesini, G, Ananyeva, L, Valentini, Gabriele, Riemekasten, G, Cutolo, M, Ionescu, R, Czirják, L, Damjanov, N, Rednic, S, and Matucci Cerinic, M.
Subjects: Adult, Male, rheumatoid arthritis, medicine.medical_specialty, Pathology, undifferentiated connective tissue disease, systemic sclerosis, autoantibodies, overlap syndrome, systemic lupus erythematosus, mixed connective tissue disease, Connective tissue, Disease, Mixed connective tissue disease, Rheumatology, medicine, Humans, Retrospective Studies, business.industry, Autoantibody, Undifferentiated connective tissue disease, Retrospective cohort study, Overlap syndrome, medicine.disease, Dermatology, Anesthesiology and Pain Medicine, medicine.anatomical_structure, Rheumatoid arthritis, Disease Progression, Female, business, Follow-Up Studies
Abstract: OBJECTIVES: To determine if mixed connective tissue disease (MCTD) can be considered an independent clinical entity, to compare 3 different classification criteria for MCTD (Kasukawa, Alarcón-Segovia, and Sharp), and to define predictors (clinical features and autoantibodies) of potential evolution toward other connective tissue diseases (CTDs). METHODS: One hundred sixty-one MCTD patients were evaluated retrospectively at the diagnosis and in 2008. They were classified, at the diagnosis, according to the 3 classification criteria of MCTD (Sharp, Alarcón-Segovia, and Kasukawa) and reclassified in 2008 according to their evolution. Statistical analyses were performed to find out predictors (clinical features and autoantibodies) of evolution into other CTDs. RESULTS: After a mean of 7.9 years of disease, 57.9% of patients still satisfied MCTD classification criteria of Kasukawa; 17.3% evolved into systemic sclerosis, 9.1% into systemic lupus erythematosus, 2.5% into rheumatoid arthritis, 11.5% was reclassified as affected by undifferentiated connective tissue disease, and 1.7% as suffering from overlap syndrome. Kasukawa's criteria were more sensitive (75%) in comparison to those of Alarcón-Segovia (73%) and Sharp (42%). The presence of anti-DNA antibodies (P = 0.012) was associated with evolution into systemic lupus erythematosus; hypomotility or dilation of esophagus (P < 0.001); and sclerodactyly (P = 0.034) with evolution into systemic sclerosis. CONCLUSIONS: MCTD is a distinct clinical entity but it is evident that a subgroup of patients may evolve into another CTD during disease progression. Initial clinical features and autoantibodies can be useful to predict disease evolution
Published: 2012

25. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author: Antonio C. Zea Mendoza, Jean Sibilia, Kamal Solanki, Cristina Mihaela Tanaseanu, Fredrick M. Wigley, Guido Valesini, M. Govoni, Lisa K. Stamp, Christopher P. Denton, Yolanda Braun-Moscovici, Ruxandra Ionescu, Øyvind Midtvedt, Ileana Nicoara, Aleksandra Stanković, Rüdiger Hein, Alina Dumitrascu, Susanne Ullman, Alan Tyndall, Sergio A. Jimenez, Irena Butrimiene, Alan Doube, Eugene J. Kucharz, Mohammed Tikly, Pier Luigi Meroni, Simon Stebbings, Renata Sokolik, Alexandra Balbir Gurman, Roger Hesselstrand, Kirsten Damgaard, Francesco Paolo Cantatore, Razvan Ionitescu, Silvana Zeni, Marco Matucci-Cerinic, Maria Rosa Pozzi, Jacques-Eric Gottenberg, Srdan Novak, Ana Maria Gherghe, David Launay, Liliana Groppa, Carlomaurizio Montecucco, Roxana Sfrent Cornateanu, Stefan Heitmann, Paloma García de la Peña Lefebvre, Daniela Opris, Peter T. Chapman, Line V. Iversen, Bernard Coleiro, Ulf Müller-Ladner, John Highton, Mara Oleszowsky, Gabriella Szücs, Magdalena Kopec-Medrek, Carlos De La Puente Buijdos, Paola Caramaschi, Magdalena Szmyrka-Kaczmarek, Rucsandra Dobrota, Gabriele Valentini, Fabiana Montoya, Blaz Rozman, Alberto Sulli, Hélène Chifflot, Raffaella Scorza, Patricia Carreira, Paulius Venalis, Lisa Maria Bambara, Torhild Garen, Isabela Tiglea, Agneta Scheja, Duska Martinovic, Jörg H W Distler, Jörg Henes, Giovanni Lapadula, Luc Mouthon, Diana Karpec, Douglas J. Veale, Valeria Riccieri, Nicolas Hunzelmann, Muriel Elhai, Serena Guiducci, Codrina Ancuta, Simonetta Pisarri, Thierry Zenone, Esthela Loyo, Branimir Anić, Claudia Günther, R. Becvar, Eugen Russu, Serena Vettori, Carlo Chizzolini, Vanessa Smith, Mengtao Li, Stanislaw Sierakowsky, Carmel Mallia, Małgorzata Widuchowska, Carolina de Souza Müller, László Czirják, Algirdas Venalis, Adrian Hij, Marta Valero Exposito, Simona Rednic, Miroslav Mayer, Laura Groseanu, Walter Alberto Sifuentes Giraldo, Murray Baron, Dominique Farge, Anna Kotulska, Marko Baresic, Svetlana Agachi, Martin Aringer, Merete Engelhart, John L. O'Donnell, Jérôme Avouac, Filip De Keyser, Ulrich A. Walker, Roberto Caporali, Harald Burkhardt, P. G. Vlachoyiannopoulos, Maurizio Cutolo, Frank A. Wollheim, Edoardo Rosato, Suzanne Kafaja, Valderílio Feijó Azevedo, Kilian Eyerich, Paolo Airò, Emmanuel Chatelus, L. Ananieva, Ira Litinsky, Andrea Lo Monaco, Vanesa Cosentino, Rita Rugiene, Eric Hachulla, P. Saar, Bojana Stamenkovic, Brigitte Krummel-Lorenz, Yannick Allanore, Elisabeth Knott, Oliver Distler, Matthias Seidel, Silvia Rodriguez Rubio, Franco Cozzi, Mihai Bojinca, Nemanja Damjanov, Maria João Salvador, Joanna Busquets, Otylia Kowal Bielecka, André Kahan, Jacek Szechiński, Daniel E. Furst, C. Mihai, Rodica Chirieac, Ewa Morgiel, Georg Schett, Armando Gabrielli, Giovanna Cuomo, Piotr Wiland, Maya N. Starovoytova, Sebastião Cezar Radominski, Gitte Strauss, Lealea Chiaburu, Florenzo Iannone, Carol M. Black, Andrea Himsel, Eduardo Kerzberg, Cecília Varjú, Vera Ortiz Santamaria, Gabriela Riemekasten, Dorota Krasowska, Marilena Gorga, Monica Popescu, Marie O'Rourke, Henrik Nielsen, Raffaele Pellerito, Ada Corrado, Elhai, M, Avouac, J, Walker, Ua, Matucci Cerinic, M, Riemekasten, G, Airò, P, Hachulla, E, Valentini, Gabriele, Carreira, Pe, Cozzi, F, Balbir Gurman, A, Braun Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller Ladner, U, Kahan, A, Distler, O, Allanore, Y, EUSTAR co, Author, EUSTAR co, Authors, Matucci-Cerinic, M, Airo, P, Valentini, G, Gurman, Ab, Braun-Moscovici, Y, Mt, Li, Muller-Ladner, U, Allanore, Y EUSTAR co-authors: Serena Guiducci, Alan, Tyndall, Giovanni, Lapadula, Florenzo, Iannone, Radim, Becvar, Stanislaw, Sierakowsky, Otylia Kowal Bielecka, Maurizio, Cutolo, Alberto, Sulli, Cuomo, Giovanna, Vettori, Serena, Simona, Rednic, Ileana, Nicoara, Vlachoyiannopoulos, P, Montecucco, C, Roberto, Caporali, Srdan, Novak, László, Czirják, Cecilia, Varju, Carlo, Chizzolini, Eugene, J Kucharz, Anna, Kotulska, Magdalena, Kopec-Medrek, Malgorzata, Widuchowska, Blaz, Rozman, Carmel, Mallia, Bernard, Coleiro, Armando, Gabrielli, Dominique, Farge, Adrian, Hij, Roger, Hesselstrand, Agneta, Scheja, Frank, Wollheim, Duska, Martinovic, Govoni, M, Andrea Lo Monaco, Nicolas, Hunzelmann, Raffaele, Pellerito, Lisa Maria Bambara, Paola, Caramaschi, Carol, Black, Christopher, Denton, Jörg, Hene, Vera Ortiz Santamaria, Stefan, Heitmann, Dorota, Krasowska, Matthias, Seidel, Mara, Oleszowsky, Harald, Burkhardt, Andrea, Himsel, Maria, J Salvador, Bojana, Stamenkovic, Aleksandra, Stankovic, Mohammed, Tikly, Maya, N Starovoytova, Merete, Engelhart, Gitte, Strau, Henrik, Nielsen, Kirsten, Damgaard, Gabriella, Szüc, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter, A Sifuentes Giraldo, Øyvind, Midtvedt, Torhild, Garen, David, Launay, Guido, Valesini, Valeria, Riccieri, Ruxandra Maria Ionescu, Daniela, Opri, Laura, Groseanu, Fredrick, M Wigley, Carmen, M Mihai, Roxana Sfrent Cornateanu, Razvan, Ionitescu, Ana Maria Gherghe, Marilena, Gorga, Rucsandra, Dobrota, Mihai, Bojinca, Georg, Schett, Jörg Hw Distler, Pierluigi, Meroni, Silvana, Zeni, Luc, Mouthon, Filip De Keyser, Vanessa, Smith, Francesco, P Cantatore, Ada, Corrado, Susanne, Ullman, Line, Iversen, Maria, R Pozzi, Kilian, Eyerich, Rüdiger, Hein, Elisabeth, Knott, Jacek, Szechinski, Piotr, Wiland, Magdalena, Szmyrka-Kaczmarek, Renata, Sokolik, Ewa, Morgiel, Brigitte, Krummel-Lorenz, Petra, Saar, Martin, Aringer, Claudia, Günther, Branimir, Anic, Marko, Baresic, Miroslav, Mayer, Sebastião, C Radominski, Carolina de Souza Müller, Valderílio, F Azevedo, Svetlana, Agachi, Liliana, Groppa, Lealea, Chiaburu, Eugen, Russu, Thierry, Zenone, Simon, Stebbing, John, Highton, Lisa, Stamp, Peter, Chapman, Murray, Baron, John, O'Donnell, Kamal, Solanki, Alan, Doube, Douglas, Veale, Marie, O'Rourke, Esthela, Loyo, Edoardo, Rosato, Simonetta, Pisarri, Cristina-Mihaela, Tanaseanu, Monica, Popescu, Alina, Dumitrascu, Isabela, Tiglea, Rodica, Chirieac, Codrina, Ancuta, Daniel, E Furst, Suzanne, Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean, Sibilia, Emmanuel, Chatelu, Jacques Eric Gottenberg, Hélène, Chifflot, Ira, Litinsky, Algirdas, Venali, Irena, Butrimiene, Paulius, Venali, Rita, Rugiene, Diana, Karpec, Eduardo, Kerzberg, Fabiana, Montoya, Vanesa, Cosentino, and Chizzolini, Carlo
Subjects: 0301 basic medicine, Male, heart dysfunction, Databases, Factual, Epidemiology, autoimmune diseases, epidemiology, systemic sclerosis, Kaplan-Meier Estimate, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Prospective Studies, Age of Onset, skin and connective tissue diseases, Prospective cohort study, ddc:616, Orvostudományok, Middle Aged, Prognosis, Connective tissue disease, 3. Good health, Europe, Cardiovascular Diseases, Cohort, Disease Progression, Female, Autoimmune Diseases, Systemic Sclerosis, Human, Adult, medicine.medical_specialty, Prognosi, Immunology, Socio-culturale, Klinikai orvostudományok, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, 03 medical and health sciences, Sex Factors, Rheumatology, Internal medicine, medicine, Humans, Sex Distribution, Systemic Sclerosi, Aged, 030203 arthritis & rheumatology, Lupus erythematosus, Scleroderma, Systemic, business.industry, medicine.disease, Pulmonary hypertension, Prospective Studie, 030104 developmental biology, Heart failure, Age of onset, business, Follow-Up Studies
Abstract: OBJECTIVES: In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. METHOD: We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. RESULTS: 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p
Published: 2014

26. The potential role of bone scintigraphy in the detection of deep muscular fascia involvement and calcinosis cutis in anti-synthetase syndrome

Author: Luisa Costa, Franco Cozzi, Mariangela Atteno, Francesco Caso, Raffaele Scarpa, Luca Cantarini, Leonardo Punzi, Paolo Sfriso, Caso, Francesco, Costa, Luisa, Atteno, Mariangela, Sfriso, P., Cozzi, F., Cantarini, L., Scarpa, Raffaele, and Punzi, L.
Subjects: Adult, Pathology, medicine.medical_specialty, Back Muscles, Calcinosis, Comorbidity, Cyclosporine, Diltiazem, Drug Therapy, Combination, Female, Humans, Myositis, Prednisolone, Skin Diseases, Technetium, Treatment Outcome, Radionuclide Imaging, Treatment outcome, Back muscles, Calcinosis cutis, Rheumatology, Drug Therapy, Medicine, Radionuclide imaging, medicine.diagnostic_test, business.industry, Muscular fascia, medicine.disease, Bone scintigraphy, Combination, business
Published: 2013

27. TGFβ receptor gene variants in systemic sclerosis-related pulmonary arterial hypertension: results from a multicentre EUSTAR study of European Caucasian patients

Author: Gilles Chiocchia, Marco Matucci-Cerinic, Otylia Kowal-Bielecka, Jean-Luc Cracowski, Ulrich A. Walker, Julien Wipff, Jörg H W Distler, Matthieu Bouaziz, László Czirják, Inga Melchers, Nicolas Hunzelmann, Elisabeth Diot, Daniele Cusi, Catherine Boileau, Sara Lupoli, Philippe Dieudé, Marc Humbert, Yannick Allanore, Giovanna Cuomo, H.-Erich Wichmann, G. Riemekasten, Ulf Müller-Ladner, Eric Hachulla, P. G. Vlachoyiannopoulos, Paola Caramaschi, Costanza Conti, Paolo Airò, Martina Müller-Nurasyid, Barbara Ruiz, Mickaël Guedj, Valeria Riccieri, Lucile Revillod, Franco Cozzi, Nemanja Damjanov, Vasiliki Kalliopi Bournia, Eugénie Koumakis, Kiet Tiev, Université Paris Descartes - Paris 5 (UPD5), Université Paris Diderot - Paris 7 (UPD7), Université d'Évry-Val-d'Essonne (UEVE), Laboratoire Statistique et Génome (SG), Institut National de la Recherche Agronomique (INRA)-Université d'Évry-Val-d'Essonne (UEVE)-Centre National de la Recherche Scientifique (CNRS), Università degli Studi di Firenze = University of Florence [Firenze] (UNIFI), Université Paris-Sud - Paris 11 (UP11), Hôpital Antoine Béclère, Assistance Publique - Hôpitaux de Paris (AP-HP), Centre Chirurgical Marie Lannelongue (CCML), Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Leibniz Association, Spedali Civil Brescia, University of Freiburg [Freiburg], Université de Lille, Droit et Santé, Università degli Studi di Milano [Milano] (UNIMI), Fdn Filarete, Partenaires INRAE, European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR), Association des Sclerodermiques de France, INSERM, Agence Nationale pour la Recherche [R07094KS], Pfizer, Koumakis, E, Wipff, J, Dieudé, P, Ruiz, B, Bouaziz, M, Revillod, L, Guedj, M, Distler, Jh, Matucci Cerinic, M, Humbert, M, Riemekasten, G, Airo, P, Melchers, I, Hachulla, E, Cusi, D, Wichmann, He, Hunzelmann, N, Tiev, K, Caramaschi, P, Diot, E, Kowal Bielecka, O, Cuomo, Giovanna, Walker, U, Czirják, L, Damjanov, N, Lupoli, S, Conti, C, Müller Nurasyid, M, Müller Ladner, U, Riccieri, V, Cracowski, Jl, Cozzi, F, Bournia, Vk, Vlachoyiannopoulos, P, Chiocchia, G, Boileau, C, Allanore, Y., Università degli Studi di Firenze = University of Florence (UniFI), and Università degli Studi di Milano = University of Milan (UNIMI)
Subjects: Genetics and Molecular Biology (all), Male, Systemic disease, Candidate gene, DNA Mutational Analysis, Biochemistry, DISEASE, Scleroderma, 0302 clinical medicine, Medizinische Fakultät, Transforming Growth Factor beta, Receptors, Immunology and Allergy, Familial Primary Pulmonary Hypertension, [MATH]Mathematics [math], skin and connective tissue diseases, 0303 health sciences, biology, integumentary system, arterial pulmonary hypertension, Pulmonary, Single Nucleotide, ASSOCIATION, Connective tissue disease, 3. Good health, Hypertension, Female, systemic sclerosi, Genotype, Hypertension, Pulmonary, European Continental Ancestry Group, Immunology, Polymorphism, Single Nucleotide, General Biochemistry, Genetics and Molecular Biology, White People, 03 medical and health sciences, Rheumatology, medicine, SNP, Humans, [INFO]Computer Science [cs], Genetic Predisposition to Disease, ddc:610, Polymorphism, HEREDITARY HEMORRHAGIC TELANGIECTASIA, Genotyping, ALK-1, 030304 developmental biology, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, MUTATIONS, Systemic, Receptors, Transforming Growth Factor beta, Biochemistry, Genetics and Molecular Biology (all), Transforming growth factor beta, medicine.disease, Pulmonary hypertension, BMPR2, biology.protein, business
Abstract: IntroductionSystemic sclerosis (SSc)-related pulmonary arterial hypertension (PAH) has emerged as a major mortality prognostic factor. Mutations of transforming growth factor beta (TGFβ) receptor genes strongly contribute to idiopathic and familial PAH.ObjectiveTo explore the genetic bases of SSc–PAH, we combined direct sequencing and genotyping of candidate genes encoding TGFβ receptor family members.Materials and methodsTGFβ receptor genes, BMPR2, ALK1, TGFR2 and ENG, were sequenced in 10 SSc–PAH patients, nine SSc and seven controls. In addition, 22 single-nucleotide polymorphisms (SNP) of these four candidate genes were tested for association in a first set of 824 French Caucasian SSc patients (including 54 SSc–PAH) and 939 controls. The replication set consisted of 1516 European SSc (including 219 SSc–PAH) and 3129 controls from the European League Against Rheumatism Scleroderma Trials and Research group network.ResultsNo mutation was identified by direct sequencing. However, two repertoried SNP, ENG rs35400405 and ALK1 rs2277382, were found in SSc–PAH patients only. The genotyping of 22 SNP including the latter showed that only rs2277382 was associated with SSc–PAH (p=0.0066, OR 2.13, 95% CI 1.24 to 3.65). Nevertheless, this was not replicated with the following result in combined analysis: p=0.123, OR 0.79, 95% CI 0.59 to 1.07.ConclusionsThis study demonstrates the lack of association between these TGFβ receptor gene polymorphisms and SSc–PAH using both sequencing and genotyping methods.
Published: 2012

28. Brief Report: Successful pregnancies but a higher risk of preterm births in patients with systemic sclerosis: An Italian multicenter study

Author: Mara, Taraborelli, Veronique, Ramoni, Antonio, Brucato, Paolo, Airo, Gianluigi, Bajocchi, Francesca, Bellisai, Domenico, Biasi, Jelena, Blagojevic, Valentina, Canti, Roberto, Caporali, Paola, Caramaschi, Ilaria, Chiarolanza, Veronica, Codullo, Franco, Cozzi, Giovanna, Cuomo, Maurizio, Cutolo, Maria De Santis, Salvatore De Vita, Emma Di Poi, Andrea, Doria, Paola, Faggioli, Maria, Favaro, Gianfranco, Ferraccioli, Clodoveo, Ferri, Rosario, Foti, Alessandro, Gerosa, Maria, Gerosa, Sarah, Giacuzzo, Leopoldo, Giani, Dilia, Giuggioli, Massimo, Imazio, Michele, Iudici, Annamaria, Iuliano, Roberto, Leonardi, Massimiliano, Limonta, Andrea, Lojacono, Chiara, Lubatti, Marco Matucci Cerinic, Antonino, Mazzone, Marianna, Meroni, Pier Luigi Meroni, Marta, Mosca, Mario, Motta, Marina, Muscara, Simona, Nava, Melissa, Padovan, Giorgio, Pagani, Giuseppe, Paolazzi, Susanna, Peccatori, Viviana, Ravagnani, Riccieri, Valeria, Rosato, Edoardo, Patrizia Rovere Querini, Salsano, Felice, Alessandro, Santaniello, Raffaella, Scorza, Chiara, Tani, Gabriele, Valentini, Valesini, Guido, Massimo, Vanoli, Barbara, Vigone, Silvana, Zeni, Angela, Tincani, Impress, Investigators, Behalf Of The Impress Investigators, O. n., Taraborelli, M, Ramoni, V, Brucato, A, Airo, P, Bajocchi, G, Bellisai, F, Biasi, D, Blagojevic, J, Canti, V, Caporali, R, Caramaschi, P, Chiarolanza, I, Codullo, V, Cozzi, F, Cuomo, G, Cutolo, M, De Santis, M, De Vita, S, Di Poi, E, Doria, A, Faggioli, P, Favaro, M, Ferraccioli, G, Ferri, C, Foti, R, Gerosa, A, Gerosa, M, Giacuzzo, S, Giani, L, Giuggioli, D, Imazio, M, Iudici, M, Iuliano, A, Leonardi, R, Limonta, M, Lojacono, A, Lubatti, C, Matucci Cerinic, M, Mazzone, A, Meroni, M, Meroni, Pl, Mosca, M, Motta, M, Muscara, M, Nava, S, Padovan, M, Pagani, G, Paolazzi, G, Peccatori, S, Ravagnani, V, Riccieri, V, Rosato, E, ROVERE QUERINI, Patrizia, Salsano, F, Santaniello, A, Scorza, R, Tani, C, Valentini, G, Valesini, G, Vanoli, M, Vigone, B, Zeni, S, and Tincani, A.
Subjects: Adult, Risk, Pediatrics, medicine.medical_specialty, Settore MED/16 - REUMATOLOGIA, systemic sclerosis, Birth weight, Immunology, Population, Intrauterine growth restriction, Scleroderma, Rheumatology, Pregnancy, Female, Fetal Growth Retardation, Humans, Infant, Newborn, Pregnancy Outcome, Premature Birth, Prevalence, Retrospective Studies, Scleroderma, Systemic, medicine, Immunology and Allergy, scleroderma, Pharmacology (medical), education, disease, education.field_of_study, pregnancies, business.industry, Systemic, Infant, Retrospective cohort study, Odds ratio, Newborn, medicine.disease, history, women, Premature birth, Gestation, pregnancy, business
Abstract: Objective To assess fetal and maternal outcomes in women with systemic sclerosis (SSc). Methods Prospectively collected data on 99 women with SSc from 25 Italian centers were analyzed retrospectively. Women with SSc were observed during 109 pregnancies (from 2000 to 2011), and outcomes were compared to those in the general obstetric population (total of 3,939 deliveries). The maternal age at conception was a mean ± SD 31.8 ± 5.3 years, and the median disease duration at conception was 60 months (range 2–193 months). Results SSc patients, compared to the general obstetric population, had a significantly increased frequency of preterm deliveries (25% versus 12%) and severe preterm deliveries (
Published: 2012

29. Italian consensus on Eular 2003 recommendations for the treatment of knee osteoarthritis

Author: A. Mannoni, Antonio Spadaro, M. Carrabba, L. Frizziero, Leonardo Punzi, L. Parente, C. Cricelli, Antonella Fioravanti, M. Chevallard, Giovanni Lapadula, Roberta Ramonda, Florenzo Iannone, E. Paresce, T. Nava, Riccardo Meliconi, Franco Cozzi, P. Patrignani, B. Canesi, Roberto Marcolongo, G. Arioli, V. Modena, Fausto Salaffi, G. Leardini, Marco A. Cimmino, V. Monteleone, Silvia Giannini, L. Molfetta, Punzi L., Canesi B., Carrabba M., Cimmino M.A., Frizziero L., Lapadula G., Arioli G., Chevallard M., Cozzi F., Cricelli C., Fioravanti A., Giannini S., Iannone F., Leardini G., Mannoni A., Meliconi R., Modena V., Molfetta L., Monteleone V., Nava T., Parente L., Paresce E., Patrignani P., Ramonda R., Salaffi F., Spadaro A., and Marcolongo R.
Subjects: musculoskeletal diseases, lcsh:Internal medicine, medicine.medical_specialty, Adrenal cortex hormones, Alternative medicine, MEDLINE, knee, lcsh:Medicine, Primary care, Patient Education as Topic, Rheumatology, Adrenal Cortex Hormones, Risk Factors, osteoarthritis of the knee, medicine, Humans, Arthroplasty, Replacement, Knee, lcsh:RC31-1245, Physical Therapy Modalities, Societies, Medical, Individual country, Analgesics, business.industry, Anti-Inflammatory Agents, Non-Steroidal, lcsh:R, treatment of osteoarthritis, Guideline, Osteoarthritis, Knee, reccomendations for osteoarthriti, Case management, Combined Modality Therapy, osteoarthritis, Italy, Antirheumatic Agents, Family medicine, recommendations, Physical therapy, osteoarthriti, business, Case Management
Abstract: The recommendations for the management of osteoarthritis (OA) of the knee firstly proposed by the EULAR in 2000, have been updated in 2003. One of the most important objectives of the expert charged to provide these recommendations was their dissemination. Thus, the information generated may be used by each individual country to produce their own set of management guidelines and algorithms for treatment in primary care. The Italian Society of Rheumatology (SIR) and the Italian League against Rheumatism (LIMAR) have organised a Consensus on the EULAR recommendations 2003 with the aim to analyse their acceptability and the applicability according to our own experience and local situations in the Italy. The results of this Consensus have demonstrated that a large majority of the EULAR recommendations are endorsed by the Italian experts. Furthermore, the final document of the Italian Consensus clearly indicated the need that the specialists involved in the management of knee OA strongly encourage the dissemination of the EULAR 2003 recommendations also in Italy.
Published: 2011

30. Serologic profile and mortality rates of scleroderma renal crisis in Italy

Author: Claudia Alpini, Gabriella Morozzi, Clodoveo Ferri, Lorenzo Cavagna, Veronica Codullo, Marco Matucci-Cerinic, Amelia Ruffatti, Serena Guiducci, Carlomaurizio Montecucco, Claudia Bonino, Franco Franceschini, Nicoletta Del Papa, Ilaria Cavazzana, Roberto Giacomelli, Gabriele Valentini, Franco Cozzi, Codullo, V, Cavazzana, I, Bonino, C, Alpini, C, Cavagna, L, Cozzi, F, DEL PAPA, N, Franceschini, F, Guiducci, S, Morozzi, G, Ruffatti, A, Ferri, C, Giacomelli, R, MATUCCI CERINIC, M, Valentini, Gabriele, and Montecucco, C.
Subjects: Adult, Male, medicine.medical_specialty, Pathology, Systemic disease, Anti-nuclear antibody, Immunology, Scleroderma Renal Crisis, Type I, Kaplan-Meier Estimate, Anti-topoisomerase I, Gastroenterology, Antibodies, Scleroderma, Serology, Scleroderma renal crisis, Anti-RNA polymerases I-III, Rheumatology, RNA Polymerase I, Antinuclear, Internal medicine, Immunopathology, medicine, Humans, Immunology and Allergy, scleroderma, Autoantibodies, Aged, Antibodies, Antinuclear, DNA Topoisomerases, Type I, Female, Italy, Kidney Diseases, Middle Aged, Prognosis, RNA Polymerase II, RNA Polymerase III, Retrospective Studies, Scleroderma, Systemic, renal crisis, business.industry, Mortality rate, Systemic, medicine.disease, Connective tissue disease, business, DNA Topoisomerases
Abstract: Objective.To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc).Methods.A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit III, and immunoprecipitation (IP) were performed.Results.Forty-six cases (38 female; 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years ± 13.2 and 55.4 years ± 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%), anti-RNAP I–III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I–III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being lower in the anti-topo I than in the anti-RNAP I–III group (p = 0.034).Conclusion.SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I–III in our patients with SRC and was associated with delayed onset and high mortality rates.
Published: 2009

31. [Prevalence and clinical significance of cathepsin G antibodies in systemic sclerosis]

Author: Maria Favaro, A. Ruffatti, Renato Alberto Sinico, Antonella Radice, Franco Cozzi, P. Grypiotis, Silvano Todesco, Marta Tonello, Grypiotis, P, Ruffatti, A, Cozzi, F, Sinico, R, Tonello, M, Radice, A, Favaro, M, and Todesco, S
Subjects: Adult, Male, lcsh:Internal medicine, Cathepsin G, lcsh:Medicine, Scleroderma, Cathepsin, Serology, chemistry.chemical_compound, Rheumatology, medicine, Prevalence, Humans, Clinical significance, lcsh:RC31-1245, skin and connective tissue diseases, Aged, Autoantibodies, Scleroderma, Systemic, integumentary system, biology, business.industry, lcsh:R, Serine Endopeptidases, Autoantibody, IIf, Middle Aged, medicine.disease, Autoantibodie, Cathepsins, chemistry, Immunology, biology.protein, Female, Antibody, business, Human
Abstract: Objectives: To evaluate the prevalence and clinical significance of cathepsin G antibodies in patients affected with systemic sclerosis (SSc, scleroderma). Methods: 115 patients affected by SSc, 55 (47,8%) with diffuse scleroderma (dSSc) and 60 (52,2%) with limited scleroderma (lSSc), were tested for cathepsin G antibodies by ELISA method. Moreover these sera were evaluated by indirect immunofluorescence (IIF) on ethanol and formalin fixed human neutrophils. Results: By means of the ELISA method 16 (13,9%) patients were found to be sera positive for anti-cathepsin G, 2 (12.5%) of which showed a perinuclear fluorescence pattern (P-ANCA) and 4 (25%) an atypical ANCA staining, while 10 (62,5%) were negative on IIF. The IIF on scleroderma sera revealed 5 (4,3%) P-ANCA and 18 (15,7%) atypical ANCA patterns. The anti-cathepsin G antibodies significantly prevailed in scleroderma sera (p=0.02) when their frequency was compared with that of healthy controls; while they were not significantly associated to any clinical or serological features of SSc patients. Conclusions: The anti-cathepsin G antibodies were significantly frequent in scleroderma sera; however, no clinical correlations were found. Thus, the significance of their presence in SSc still needs to be clarified.
Published: 2004

32. European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres

Author: R. Becvar, Murat Inanc, R. Scorza, E. Tirri, Salvatore D'Angelo, Walter Bencivelli, Jill J. F. Belch, H. Nielsen, P. Bruhlman, A. J. Silman, Panayiotis G. Vlachoyiannopoulos, Clodoveo Ferri, N. J. McHugh, F.H.J. van den Hoogen, G. Hayem, L. Czirják, B. Dziankowska, Franco Cozzi, A. A. Drosos, A. Gabrielli, M. Matucci Cerinic, G. Valentini, A. Della Rossa, C. M. Black, Stefano Bombardieri, Roberto Giacomelli, DELLA ROSSA, A, Valentini, Gabriele, Bombardieri, S, Bencivelli, W, Silman, Aj, D'Angelo, S, Cerinic, Mm, Belch, Jf, Black, Cm, Becvar, R, Bruhlman, P, Cozzi, F, Czirjak, L, Drosos, Aa, Dziankowska, B, Ferri, C, Gabrielli, A, Giacomelli, R, Hayem, G, Inanc, M, Mchugh, Nj, Nielsen, H, Scorza, R, Tirri, E, VAN DEN HOOGEN, Fh, and Vlachoyiannopoulos, Pg
Subjects: Adult, Male, medicine.medical_specialty, Time Factors, Adolescent, Referral, Immunology, Disease, General Biochemistry, Genetics and Molecular Biology, Europe/epidemiology, Scleroderma, Systemic/complications/*diagnosis/drug therapy/*epidemiology, Age Distribution, Rheumatology, Internal medicine, Epidemiology, Prevalence, medicine, Humans, Immunology and Allergy, Prospective Studies, Sex Distribution, Child, Prospective cohort study, Aged, Aged, 80 and over, Scleroderma, Systemic, business.industry, activity criteria for systemic sclerosis, Public health, Attendance, Middle Aged, medicine.disease, Connective tissue disease, Surgery, Extended Report, Europe, Family medicine, Female, business
Abstract: OBJECTIVE: To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches. METHODS: In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enroll consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed. RESULTS: Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease. CONCLUSIONS: The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease. Ann Rheum Dis
Published: 2001

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32 results on '"Cozzi F"'

1. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

2. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

3. What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

4. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database

5. Effect of Macitentan on the Development of New Ischemic Digital Ulcers in Patients With Systemic Sclerosis

6. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database

7. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

8. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

9. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

10. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients

11. VENTRICULAR ARRHYTHMIAS AND SUDDEN DEATH IN ATHLETES

12. CT for assessing feasibility of separation of thoracopagus twins

13. Induction of supraventricular tachyarrhythmia at rest and during exercise with transoesophageal atrial pacing in the electrophysiological evaluation of asymptomatic athletes with Wolff-Parkinson-White syndrome

14. Impaired immunogenicity to COVID-19 vaccines in autoimmune systemic diseases. High prevalence of non-response in different patients' subgroups

15. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

16. COVID-19 and systemic sclerosis: clinicopathological implications from Italian nationwide survey study

17. Serum anti-GM2 and anti-GalNAc-GD1a IgG antibodies are biomarkers for acute canine polyradiculoneuritis

18. Comparison of intranasal versus intravenous midazolam for management of status epilepticus in dogs: A multi‐center randomized parallel group clinical study

19. Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments

20. Agreement of surgeon's perception of the effectiveness of spinal cord decompression with findings on postoperative magnetic resonance imaging for dogs surgically treated for intervertebral disk extrusion

21. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

22. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis

23. Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients

24. 'To Be or Not To Be,' Ten Years After: Evidence for Mixed Connective Tissue Disease as a Distinct Entity

25. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

26. The potential role of bone scintigraphy in the detection of deep muscular fascia involvement and calcinosis cutis in anti-synthetase syndrome

27. TGFβ receptor gene variants in systemic sclerosis-related pulmonary arterial hypertension: results from a multicentre EUSTAR study of European Caucasian patients

28. Brief Report: Successful pregnancies but a higher risk of preterm births in patients with systemic sclerosis: An Italian multicenter study

29. Italian consensus on Eular 2003 recommendations for the treatment of knee osteoarthritis

30. Serologic profile and mortality rates of scleroderma renal crisis in Italy

31. [Prevalence and clinical significance of cathepsin G antibodies in systemic sclerosis]

32. European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres

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