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201. The role of extra-corporeal membrane oxygenation (ECMO) in the treatment of diffuse alveolar haemorrhage secondary to ANCA-associated vasculitis: report of two cases and review of the literature

Author

Delvino, P, Monti, S, Balduzzi, S, Belliato, M, Montecucco, C, Caporali, R, Delvino, P, Monti, S, Balduzzi, S, Belliato, M, Montecucco, C, and Caporali, R

Abstract

Diffuse alveolar haemorrhage (DAH) secondary to anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a rare life-threatening condition presenting with severe respiratory failure. The management of AAV-related DAH consists of remission induction immunosuppressive therapy, which requires time to be effective, with significant fatality rates despite appropriate treatment. Extracorporeal membrane oxygenation (ECMO) can support gas exchanges providing the time necessary for immunosuppressive treatment to control the underlying disease in cases refractory to the conventional ventilation techniques. Despite severe preexisting bleeding has been considered a relative contraindication, ECMO has proven to be life-saving in several cases of respiratory failure associated with pulmonary haemorrhage due to various causes, including AAV. We reviewed the clinical presentation and course of two patients affected by AAV-related DAH treated at our Institution between 2012 and 2017, whose management required the use of veno-venous ECMO. We reviewed the current literature on the role of ECMO in the support of these patients. In both patients, ECMO provided life support and allowed disease control, in combination with immunosuppressive treatment. Despite systemic anticoagulation, clinical improvement was achieved without exacerbation of the pulmonary bleeding. We performed a literature review, and summarized available data confirming the effectiveness and safety of ECMO in AAV-related DAH. ECMO has a life-saving role in the management of patients with severe respiratory failure due to ANCA-associated pulmonary capillaritis.

Published
2019

202. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel

Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

Published
2019

203. Long-term retention rate of anakinra in adult onset Still’s disease and predictive factors for treatment response.

Author

Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

We have evaluated long-term retention rate of anakinra in adult onset Still disease and predictive factors for treatment response. The risk of losing anakinra efficacy increases along with the number of swollen joints at the start of therapy, while the typical skin rash is a negative predictor of anakinra withdrawal.

Published
2019

208. Cellular vacuoles induced by Helicobacter pylori originate from late endosomal compartments

Author

Papini, E., Bernard, M. De, Milia, E., Bugnoli, M., Zerial, M., Rappuoli, R., and Montecucco, C.

Subjects
Helicobacter pylori -- Research, Epithelial cells -- Physiological aspects, Science and technology
Abstract

Characterization of the vacuoles caused by pathogenic strains of Helicobacter pylori through the distribution of markers of several membrane traffic compartments that include early endosomes, late endosomes such as rab7, cathepsin D, TGN and Lucifer yellow reveals that only anti-rab7 antibodies stain the vacuoles stimulated by H. pylori, indicating their origin in the late endosomal compartments. Vacuolar membranes exhibit an increase in the labeling of rab7 as vacuolation proceeds, unaccompanied by a simultaneous rise in cellular rab7 levels.

Published
1994

209. 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
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213. Development and First Validation of a Disease Activity Score for Gout

Author

Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scire C. A., Carrara G., Viroli C., Cimmino M. A., Taylor W. J., Manara M., Govoni M., Salaffi F., Punzi L., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., Veneto V., Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scire C. A., Carrara G., Viroli C., Cimmino M. A., Taylor W. J., Manara M., Govoni M., Salaffi F., Punzi L., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., and Veneto V.

Abstract

Objective: To develop a new composite disease activity score for gout and provide its first validation. Methods: Disease activity has been defined as the ongoing presence of urate deposits that lead to acute arthritis and joint damage. Every measure for each Outcome Measures in Rheumatology core domain was considered. A 3-step approach (factor analysis, linear discriminant analysis, and linear regression) was applied to derive the Gout Activity Score (GAS). Decision to change treatment or 6-month flare count were used as the surrogate criteria of high disease activity. Baseline and 12-month followup data of 446 patients included in the Kick-Off of the Italian Network for Gout cohort were used. Construct- and criterion-related validity were tested. External validation on an independent sample is reported. Results: Factor analysis identified 5 factors: patient-reported outcomes, joint examination, flares, tophi, and serum uric acid (sUA). Discriminant function analysis resulted in a correct classification of 79%. Linear regression analysis identified a first candidate GAS including 12-month flare count, sUA, visual analog scale (VAS) of pain, VAS global activity assessment, swollen and tender joint counts, and a cumulative measure of tophi. Alternative scores were also developed. The developed GAS demonstrated a good correlation with functional disability (criterion validity) and discrimination between patient- and physician-reported measures of active disease (construct validity). The results were reproduced in the external sample. Conclusion: This study developed and validated a composite measure of disease activity in gout. Further testing is required to confirm its generalizability, responsiveness, and usefulness in assisting with clinical decisions.

Published
2016

214. Undiagnosed connective tissue diseases High prevalence in pulmonary arterial hypertension patients

Author

Cavagna, L, Codullo, V, Ghio, S, Scire, C, Guzzafame, E, Scelsi, L, Rossi, S, Montecucco, C, Caporali, R, Cavagna L., Codullo V., Ghio S., Scire C. A., Guzzafame E., Scelsi L., Rossi S., Montecucco C., Caporali R., Cavagna, L, Codullo, V, Ghio, S, Scire, C, Guzzafame, E, Scelsi, L, Rossi, S, Montecucco, C, Caporali, R, Cavagna L., Codullo V., Ghio S., Scire C. A., Guzzafame E., Scelsi L., Rossi S., Montecucco C., and Caporali R.

Abstract

Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory. To estimate frequency and prognosis of previously undiagnosed CTDs in a suspect idiopathic (i) PAH cohort. Consecutive patients with PAH confirmed by right heart catheterization referred at the Cardiology Division of our Hospital without a previous rheumatological assessment or the occurrence of other conditions explaining PAH were checked for CTD by a clinical, laboratory, and instrumental evaluation. Survival in each group has also been analyzed. In our study 17 of 49 patients were classified as CTD-PAH, corresponding to a prevalence (95% CI) of 34.7% (21.7-49.6%). ANA positivity had 94% (71.3-99.9%) sensitivity and 78.1% (60-90.7%) specificity for a diagnosis of CTD-PAH; Raynaud phenomenon (RP) showed 83.3% (51.6-97.9%) sensitivity and 100% (90.5-100%) specificity for the diagnosis of Systemic Sclerosis (SSc)-PAH. At diagnosis, SSc patients were older and had a lower creatinine clearance compared with iPAH and other CTD-PAH. After a median follow-up of 44 (2-132) months, 18 of 49 (36.7%) patients died: 31.2% in the iPAH group, 20% in the CTD-, and 58.3% in the SSc-PAH group. Mortality was significantly higher in SSc-PAH (HR 3.32, 1.11-9.95, P<0.05) versus iPAH. We show a high prevalence of undiagnosed CTDs in patients with iPAH without a previous rheumatological assessment. All patients with RP were diagnosed with SSc. Our data stress the importance of a rheumatological assessment in PAH, especially because of the unfavorable prognostic impact of an associated SSc.

Published
2016

215. Cardiovascular comorbidities relate more than others with disease activity in rheumatoid arthritis

Author

Crepaldi, G, Scire, C, Carrara, G, Sakellariou, G, Caporali, R, Hmamouchi, I, Dougados, M, Montecucco, C, Crepaldi G., Scire C. A., Carrara G., Sakellariou G., Caporali R., Hmamouchi I., Dougados M., Montecucco C., Crepaldi, G, Scire, C, Carrara, G, Sakellariou, G, Caporali, R, Hmamouchi, I, Dougados, M, Montecucco, C, Crepaldi G., Scire C. A., Carrara G., Sakellariou G., Caporali R., Hmamouchi I., Dougados M., and Montecucco C.

Abstract

Objectives. To explore the influence of comorbidities on clinical outcomes and disease activity in rheumatoid arthritis (RA). Methods. In patients included in the cross-sectional observational multicenter international study COMORA, demographics, disease characteristics and comorbidities (hypertension, diabetes, hyperlipidemia, renal failure, ischemic heart disease, stroke, cancer, gastro-intestinal ulcers, hepatitis, depression, chronic pulmonary disease, obesity) were collected. Multivariable linear regression models explored the relationship between each comorbidity and disease activity measures: 28-swollen joint count (SJC), 28-tender joint count (TJC), erythrocyte sedimentation rate (ESR), patient's and physician's global assessment (PtGA, PhGA), patient reported fatigue and 28-Disease Activity Score (DAS28). Results are expressed as mean difference (MD) adjusted for the main confounders (age, gender, disease characteristics and treatment). Results. A total of 3,920 patients were included: age (mean ±SD) 56.27 ±13.03 yrs, female 81.65%, disease duration median 7.08 yrs (IQR 2.97-13.27), DAS28 (mean ±SD) 3.74 ± 1.55. Patients with diabetes had more swollen and tender joints and worse PtGA and PhGA (MD +1.06, +0.93, +0.53 and +0.54, respectively). Patients with hyperlipidemia had a lower number of swollen and tender joints, lower ESR and better PtGA and PhGA (MD -0.77, -0.56, -3.56, -0.31 and -0.35, respectively). Patients with history of ischemic heart disease and obese patients had more tender joints (MD +1.27 and +1.07) and higher ESR levels (MD +5.64 and +5.20). DAS28 is influenced exclusively by cardiovascular comorbidities, in particular diabetes, hyperlipidemia, ischemic heart disease and obesity. Conclusions. Cardiovascular comorbidities relate more than others with disease activity in RA. Diabetes and hyperlipidemia in particular seem associated with higher and lower disease activity respectively influencing almost all considered outcomes, suggesting a s

Published
2016

217. Clinical spectrum time course in anti jo-1 positive antisynthetase syndrome: Results from an international retrospective multicenter study

Author

Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., Rosenthal K., Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, AENEAS (American, European NEtwork of Antisynthetase Syndrome) collaborative group, [Cavagna,L, Caporali,L, Montecucco,C] Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. [Nuño,L] Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. [Scire,CA] Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy. [Govoni ,M, Furini,F, La Corte,R, Foschi,V] UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. [Lopez Longo.FJ, Martínez-Barrio,J, Hinojosa,M] Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. [Franceschini,F, Airó,P, Cavazzana,I] Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. [Neri,R, Barsotti,S] Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. [Castañeda,S] Department of Rheumatology, Hospital Universitario de la Princesa, IIS Princesa, Madrid, Spain.[Sifuentes Giraldo,WA, Bachiller Corral,AJ] Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. [Iannone,F] Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. [Fusaro,E, Parisi,S] Department of Rheumatology, Città Della Salute e della Scienza, Torino, Italy. [Paolazzi,G, Barausse,G, Bortolotti,R] Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. [Pellerito,R, Vitetta,R, Russo,A] Division of Rheumatology, Mauriziano Hospital, Turin, Italy. [Schwarting,A, Menke,J] Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. [Saketkoo,LA] Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA. [Ortego-Centeno,N] Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. [Quartuccio,L] Santa Maria della Misericordia Hospital, Udine, Italy. [Bartoloni,E] Clinic of Rheumatology, Department of Medical and Biological Sciences. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. [Specker,C] Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. [Pina Murcia,T, and González-Gay,MA] Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. [Triantafyllias,K] ACURA Rheumatology Center, Bad Kreuznach, Germany. [Bajocchi,G] Rheumatology Unit, Department of Internal Medicine, S. Maria Hospital—IRCCS, Reggio Emilia, Italy. [Bravi,E] Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. [Selmi,C] Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy.

Subjects
Male, Pathology, Neurology, Anti Jo-1, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective Studies [Medical Subject Headings], Medizin, Arthritis, Antisynthetase syndrome, Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], Antinuclear, Masculino, Myositis, Persons::Persons::Age Groups::Adult::Aged [Medical Subject Headings], Medicine (all), Interstitial lung disease, Femenino, General Medicine, Middle Aged, Diseases::Musculoskeletal Diseases::Muscular Diseases::Myositis [Medical Subject Headings], Humanos, Anticuerpos antinucleares, Antibodies, Antinuclear, Diseases::Musculoskeletal Diseases::Joint Diseases::Arthritis [Medical Subject Headings], Female, Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Autoantibodies::Antibodies, Antinuclear [Medical Subject Headings], Adult, medicine.medical_specialty, Check Tags::Male [Medical Subject Headings], Antibodies, NO, Estudios retrospectivos, Internal medicine, medicine, Humans, Risk factor, Aged, Retrospective Studies, Persons::Persons::Age Groups::Adult [Medical Subject Headings], Artritis, business.industry, Retrospective cohort study, Persons::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings], Anti Jo-1, Antisynthetase Syndrome, medicine.disease, Dermatology, Rheumatology, Check Tags::Female [Medical Subject Headings], Miositis, antisynthetase syndrome, business
Abstract

Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestations in patients with incomplete forms is common, thus indicating the need for an adequate clinical and instrumental follow-up. Furthermore, the study clearly suggested that in ASSD multidisciplinary approach involving Rheumatology, Neurology, Pneumology, and Internal Medicine specialists is mandatory. CA extern

Published
2015

218. P6478Resting and exercise pulmonary artery systolic pressure to rule out later development of pulmonary arterial hypertension in systemic sclerosis

Author

Gargani, L, primary, Codullo, V, additional, Argiento, P, additional, Moreo, A, additional, Pieri, F, additional, Mori, F, additional, Epis, O, additional, Moggi-Pignone, A, additional, Montecucco, C, additional, Valentini, G, additional, Matucci-Cerinic, M, additional, Sicari, R, additional, Ghio, S, additional, Bossone, E, additional, and D'Alto, M, additional

Published
2019
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219. The booming field of botulinum neurotoxins

Author

Rossetto, O., primary, Pirazzini, M., additional, Zanetti, G., additional, Azarnia Tehran, D., additional, Lista, F., additional, Binz, T., additional, Shone, C.S., additional, Paradisi, C., additional, Mattarei, A., additional, and Montecucco, C., additional

Published
2019
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221. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai C, Landewé R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM, Ionescu R, Rednic S, Allanore Y, Avouac J, Czirják L, Hachulla E, Riemekasten G, Cozzi F, Airò P, Cutolo M, Mueller-Ladner U1 Matucci-Cerinic M, Launay D, Dobrotă R, Sfrenţ-Cornăţeanu R, Zingarelli S, Pigatto E, Cuomo G, Caramaschi P, Ananieva L, Ullman S, Iversen L, Gurman AB, Braun-Moscovici Y, Carreira PE, Joven BE, Minier T, Guiducci S, Bellando-Randone S, Pellerito R, Hunzelmann N, Tarner IH, Radominski SC, de Souza Müller C, Iannone F, Henes J, Bancel DF, Damjanov N, Ostojić P, Pozzi MR, Hesselstrand R, Denton C, Krasowska D, Tikly M, Riccieri V, Cantatore FP, Corrado A, Da Silva JA, Salvador MJ, Tyndall A, Gabrielli A, Distler O, Jordan S, Heitmann S, Burkhardt H, Himsel A, Rozman B, Smith V, De Keyser F, Kalitena DM, Radic M, Filipescu I, Petcu A, Vlachoyiannopoulos P, Kucharz EJ, Widuchowska M, Kopec-Medrek M, Kotulska A, Szücs G, Stankovic A, Stamenkovic B, Selmi CF, De Santis M, Marasini B, Coleiro B, Santamaria VO, Westhovens R, Bečvář R, Novak S, Engelhart M, Meroni P, Ingegnoli F, Zeni S, Sulli A, Distler J, Yavuz S, Montecucco C, Eyerich K, Krummel-Lorenz B, Zenone T, Midtvedt Ø, Chizzolini C, Seidel M, Oleszowsky M, Üprus M, Opriş D, Groşeanu L, Bielecka OK, Antonio ZM, Szechinski J, Morović-Vergles J, Scorza R, Puppo F, Mathieu A, Anic B, Stork J, Stebbings S, Inanc M, Hasler P, von Mühlen CA, Aringer M, Popa S, Li M, Rosato E., Mihai, C, Landewé, R, van der Heijde, D, Walker, Ua, Constantin, Pi, Gherghe, Am, Ionescu, R, Rednic, S, Allanore, Y, Avouac, J, Czirják, L, Hachulla, E, Riemekasten, G, Cozzi, F, Airò, P, Cutolo, M, Mueller-Ladner U1 Matucci-Cerinic, M, Launay, D, Dobrotă, R, Sfrenţ-Cornăţeanu, R, Zingarelli, S, Pigatto, E, Cuomo, G, Caramaschi, P, Ananieva, L, Ullman, S, Iversen, L, Gurman, Ab, Braun-Moscovici, Y, Carreira, Pe, Joven, Be, Minier, T, Guiducci, S, Bellando-Randone, S, Pellerito, R, Hunzelmann, N, Tarner, Ih, Radominski, Sc, de Souza Müller, C, Iannone, F, Henes, J, Bancel, Df, Damjanov, N, Ostojić, P, Pozzi, Mr, Hesselstrand, R, Denton, C, Krasowska, D, Tikly, M, Riccieri, V, Cantatore, Fp, Corrado, A, Da Silva, Ja, Salvador, Mj, Tyndall, A, Gabrielli, A, Distler, O, Jordan, S, Heitmann, S, Burkhardt, H, Himsel, A, Rozman, B, Smith, V, De Keyser, F, Kalitena, Dm, Radic, M, Filipescu, I, Petcu, A, Vlachoyiannopoulos, P, Kucharz, Ej, Widuchowska, M, Kopec-Medrek, M, Kotulska, A, Szücs, G, Stankovic, A, Stamenkovic, B, Selmi, Cf, De Santis, M, Marasini, B, Coleiro, B, Santamaria, Vo, Westhovens, R, Bečvář, R, Novak, S, Engelhart, M, Meroni, P, Ingegnoli, F, Zeni, S, Sulli, A, Distler, J, Yavuz, S, Montecucco, C, Eyerich, K, Krummel-Lorenz, B, Zenone, T, Midtvedt, Ø, Chizzolini, C, Seidel, M, Oleszowsky, M, Üprus, M, Opriş, D, Groşeanu, L, Bielecka, Ok, Antonio, Zm, Szechinski, J, Morović-Vergles, J, Scorza, R, Puppo, F, Mathieu, A, Anic, B, Stork, J, Stebbings, S, Inanc, M, Hasler, P, von Mühlen, Ca, Aringer, M, Popa, S, Li, M, and Rosato, E.

Subjects
Epidemiology, Cardiovascular Disease, Systemic Sclerosis
Published
2016

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

Author

Iannone F., 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., Kowal-Bielecka O., Proudman S.M., Huq M., Stevens W., Wilson M.E., Sahhar J., Baron M., Hudson M., Allanore Y., Distler O., Bielecka O.K., 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., Pope 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., 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., Iannone F., 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., Kowal-Bielecka O., Proudman S.M., Huq M., Stevens W., Wilson M.E., Sahhar J., Baron M., Hudson M., Allanore Y., Distler O., Bielecka O.K., 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., Pope 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., 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., and Bojinca M.

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. Method(s): 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. Result(s): 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%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis (all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusion(s): 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.Copyright © 2017 Wichtig International

Published
2018

223. Patients’, physicians’, nurses’, and pharmacists’ preferences on the characteristics of biologic agents used in the treatment of rheumatic diseases

Author

Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, Mecchia, Monica, Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, and Mecchia, Monica

Abstract

Objective: To estimate preferences in relevant treatment characteristics evaluated by different groups involved in the management of patients with rheumatic diseases. Subjects and methods: We surveyed patients with rheumatic diseases, and rheumatologists, nurses, and pharmacists with experience in treatment with/provision of biologic drugs for these patients. Through a discrete choice experiment, participants evaluated 16 possible scenarios in which pairs of similarly efficacious treatments were described with six characteristics: 1) frequency of administration; 2) mode and place of administration; 3) manner, helpfulness, efficiency, and courtesy of health personnel; 4) frequency of reactions at the site of drug administration; 5) severity of generalized undesired/allergic reactions; and 6) additional cost. The direction and strength of preferences toward each characteristic level and the relative importance of each characteristic were estimated through a random-effects conditional logistic regression model. Results: In total, 513 patients, 110 rheumatologists, 51 nurses, and 46 pharmacists from 30 centers in Italy participated. Characteristics 3, 4, and 6 were the most important for every subgroup; 1 was least important for patients and rheumatologists, 2 was least important for pharmacists, and 2 and 5 were least important for nurses. For characteristic 2, pharmacists preferred subcutaneous self-injection with a syringe; nurses preferred assisted infusion at an infusion center close to the patient’s home; patients and rheumatologists preferred subcutaneous self-injection with a pen. Conclusion: The different preferences for some characteristics shown by the different groups can play an important role, together with purely clinical aspects, in the choice and consequent benefit of treatments, contributing also to a more satisfactory use of resources

Published
2018

224. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

Author

González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, Cavagna, Lorenzo, González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, and Cavagna, Lorenzo

Abstract

Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Published
2018

225. A validation study of a new classification algorithm to identify rheumatoid arthritis using administrative health databases: Case-control and cohort diagnostic accuracy studies. Results from the RECord linkage on Rheumatic Diseases study of the Italian Society for Rheumatology

Author

Carrara, G, Scirè, C, Zambon, A, Cimmino, M, Cerra, C, Caprioli, M, Cagnotto, G, Nicotra, F, Arfè, A, Migliazza, S, Corrao, G, Minisola, G, Montecucco, C, ZAMBON, ANTONELLA, NICOTRA, FEDERICA, CORRAO, GIOVANNI, Montecucco, C., Carrara, G, Scirè, C, Zambon, A, Cimmino, M, Cerra, C, Caprioli, M, Cagnotto, G, Nicotra, F, Arfè, A, Migliazza, S, Corrao, G, Minisola, G, Montecucco, C, ZAMBON, ANTONELLA, NICOTRA, FEDERICA, CORRAO, GIOVANNI, and Montecucco, C.

Abstract

Objectives: To develop and validate a new algorithm to identify patients with rheumatoid arthritis (RA) and estimate disease prevalence using administrative health databases (AHDs) of the Italian Lombardy region. Design: Case-control and cohort diagnostic accuracy study. Methods: In a randomly selected sample of 827 patients drawn from a tertiary rheumatology centre (training set), clinically validated diagnoses were linked to administrative data including diagnostic codes and drug prescriptions. An algorithm in steps of decreasing specificity was developed and its accuracy assessed calculating sensitivity/specificity, positive predictive value (PPV)/negative predictive value, with corresponding CIs. The algorithm was applied to two validating sets: 106 patients from a secondary rheumatology centre and 6087 participants from the primary care. Alternative algorithms were developed to increase PPV at population level. Crude and adjusted prevalence estimates taking into account algorithm misclassification rates were obtained for the Lombardy region. Results: The algorithms included: RA certification by a rheumatologist, certification for other autoimmune diseases by specialists, RA code in the hospital discharge form, prescription of disease-modifying antirheumatic drugs and oral glucocorticoids. In the training set, a four-step algorithm identified clinically diagnosed RA cases with a sensitivity of 96.3 (95% CI 93.6 to 98.2) and a specificity of 90.3 (87.4 to 92.7). Both external validations showed highly consistent results. More specific algorithms achieved >80% PPV at the population level. The crude RA prevalence in Lombardy was 0.52%, and estimates adjusted for misclassification ranged from 0.31% (95% CI 0.14% to 0.42%) to 0.37% (0.25% to 0.47%). Conclusions: AHDs are valuable tools for the identification of RA cases at the population level, and allow estimation of disease prevalence and to select retrospective cohorts.

Published
2015

226. Lifestyle and dietary habits of patients with gout followed in rheumatology settings

Author

Manara, M, Carrara, G, Scire, C, Cimmino, M, Govoni, M, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Manara M., Carrara G., Scire C. A., Cimmino M. A., Govoni M., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., Veneto V., Manara, M, Carrara, G, Scire, C, Cimmino, M, Govoni, M, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Manara M., Carrara G., Scire C. A., Cimmino M. A., Govoni M., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., and Veneto V.

Abstract

Diet and lifestyles modification are core aspects of the non-pharmacological management of gout, but a poor consistency with suggested guidelines is reported. This study aimed to investigate dietary and lifestyle habits of patients with gout followed in rheumatology settings. Data were retrieved from the baseline dataset of the KING study, a multicentre cohort study of patients with gout followed in rheumatology settings. Dietary habits were assessed with the Italian National Institute of Statistics (ISTAT) food-frequency questionnaire and compared with reported data about general population. The relative increase of exposure was estimated by standardized prevalence ratios adjusted for gender, age and geographical distribution. The study population included 446 patients, with a mean age of 63.9 years and a M/F ratio of 9:1. Compared to the Italian population, gouty patients showed a higher prevalence of obesity [1.82 (1.52-2.18)] and a higher consumption of wine [1.85 (1.48-2.32)] and beer [2.21 (1.68-2.90)], but a lower prevalence of smoking and a lower intake of liquor. They showed a lower intake of red meat [0.80 (0.71-0.91)], but a similar intake of other tested dietary factors. Gouty patients’ lifestyle is still partially different from the recommended.

Published
2015

227. Clinical spectrum time course in anti jo-1 positive antisynthetase syndrome: Results from an international retrospective multicenter study

Author

Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., Rosenthal K., Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., and Rosenthal K.

Abstract

Anti Jo-1 antibodies are the main markers of the anti-synthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestati

Published
2015

236. FRI0373 Long-term follow-up of 320 children born to mothers with systemic autoimmune diseases: a multicentre italian survey from 24 rheumatology centres

Author

Lazzaroni, M.G., primary, Nalli, C., additional, Andreoli, L., additional, Carini, C., additional, Bartoloni-Bocci, E., additional, Gerli, R., additional, Chighizola, C.B., additional, Gerosa, M., additional, Meroni, P.L., additional, Sinigaglia, L., additional, Conigliaro, P., additional, Perricone, R., additional, Corrado, A., additional, Cantatore, F., additional, D’Angelo, S., additional, Favaro, M., additional, Larosa, M., additional, Doria, A., additional, Ruffatti, A., additional, Generali, E., additional, Selmi, C., additional, Meroni, M., additional, Cutolo, M., additional, Padovan, M., additional, Govoni, M., additional, Pazzola, G., additional, Salvarani, C., additional, Peccatori, S., additional, Paolazzi, G., additional, Prevete, I., additional, Sebastiani, G.D., additional, Minisola, G., additional, Brucato, A., additional, Ramoni, V., additional, Caporali, R., additional, Montecucco, C., additional, Tani, C., additional, Signorini, V., additional, Mosca, M., additional, Trevisani, M., additional, Malavolta, N., additional, Vadacca, M., additional, Afeltra, A., additional, Vivaldelli, E., additional, Maier, A., additional, Visalli, E., additional, Foti, R., additional, Zuliani, L., additional, Gabrielli, A., additional, Campochiaro, C., additional, Baldissera, E., additional, Sabbadini, M.G., additional, Romeo, N., additional, and Tincani, A., additional

Published
2018
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242. PS4:79 Long-term follow-up of 320 chilren born to mothers with systemic autoimmune diseases: a multicentre survey from 24 rheumatology centres in italy

Author

Lazzaroni, MG, primary, Nalli, C, additional, Andreoli, L, additional, Carini, C, additional, Dall’Ara, F, additional, Rodrigues, M, additional, Bartoloni Bocci, E, additional, Gerli, R, additional, Chighizola, CB, additional, Gerosa, M, additional, Meroni, PL, additional, Sinigaglia, L, additional, Conigliaro, P, additional, Perricone, R, additional, Corrado, A, additional, Cantatore, F, additional, D’Angelo, S, additional, Favaro, M, additional, Larosa, M, additional, Doria, A, additional, Ruffatti, A, additional, Generali, E, additional, Selmi, C, additional, Meroni, M, additional, Cutolo, M, additional, Padovan, M, additional, Govoni, M, additional, Pazzola, G, additional, Salvarani, C, additional, Peccatori, S, additional, Prevete, I, additional, Minisola, G, additional, Sebastiani, GD, additional, Brucato, A, additional, Ramoni, V, additional, Caporali, R, additional, Montecucco, C, additional, Tani, C, additional, Signorini, V, additional, Mosca, M, additional, Trevisani, M, additional, Malavolta, N, additional, Vadacca, M, additional, Afeltra, A, additional, Vivaldelli, E, additional, Maier, A, additional, Visalli, E, additional, Foti, R, additional, Zuliani, L, additional, Gabrielli, A, additional, Campochiaro, C, additional, Baldissera, E, additional, Sabbadini, MG, additional, Romeo, N, additional, and Tincani, A, additional

Published
2018
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