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2. Reliability and Minimal Clinically Important Differences of FVC. Results from the Scleroderma Lung Studies (SLS-I and SLS-II)

Author

Kafaja, Suzanne, Clements, Philip J, Wilhalme, Holly, Tseng, Chi-hong, Furst, Daniel E, Kim, Grace Hyun, Goldin, Jonathan, Volkmann, Elizabeth R, Roth, Michael D, Tashkin, Donald P, Khanna, Dinesh, Arbor, Ann, Khanna, D, Angeles, Los, Clements, PJ, Tashkin, DP, Elashoff, R, Goldin, J, Roth, M, Furst, D, Bulpitt, K, Chung, W-LJ, Viasco, S, Sterz, M, Woolcock, L, Yan, X, Ho, J, Vasunilashorn, S, da Costa, I, Seibold, JR, Riley, DJ, Amorosa, JK, Hsu, VM, McCloskey, DA, Wilson, JE, Varga, J, Schraufnagel, D, Wilbur, A, Lapota, D, Arami, S, Cole-Saffold, P, Simms, R, Theodore, A, Clarke, P, Korn, J, Tobin, K, Nuite, M, Silver, R, Bolster, M, Strange, C, Schabel, S, Smith, E, Arnold, J, Caldwell, K, Bonner, M, Wise, R, Wigley, F, White, B, Hummers, L, Bohlman, M, Polito, A, Leatherman, G, Forbes, E, Daniel, M, Steen, V, Read, C, Cooper, C, Wheaton, S, Carey, A, Ortiz, A, Mayes, M, Parsley, E, Oldham, S, Filemon, T, Jordan, S, Perry, M, K. Connolly, Golden, J, Wolters, P, Webb, R, Davis, J, Antolos, C, Maynetto, C, Fessler, B, Olman, M, Sanders, C, Heck, L, Parkhill, T, Rothfield, N, Metersky, M, Cobb, R, Aberles, M, Ingenito, F, Breen, E, Mubarak, K, Granda, JL, Silva, J, Injic, Z, and Alexander, R

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Scleroderma, Lung, Clinical Research, Autoimmune Disease, Cohort Studies, Disease Progression, Female, Humans, Lung Diseases, Interstitial, Male, Middle Aged, Minimal Clinically Important Difference, Reproducibility of Results, Scleroderma, Systemic, Vital Capacity, interstitial lung disease, systemic sclerosis, FVC%, minimal clinically important differences, patient-reported outcomes, Medical and Health Sciences, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

ObjectivesTo assess the reliability and the minimal clinically important differences (MCID) for FVC% predicted in the Scleroderma Lung Study I and II.MethodsUsing data from SLS I and II (N=300), we evaluated the test-retest reliability for FVC% predicted (FVC%; screening vs. baseline) using intra-class correlation (ICC). MCID estimates at 12 months were calculated in the pooled cohort (SLS-I and II) using 2 anchors: Transition Dyspnea Index (≥change of 1.5 units for improvement and worsening, respectively) and the SF-36 Health Transition question: "Compared to one year ago, how would you rate your health in general now?", where "somewhat better" or "somewhat worse" were defined as the MCID estimates. We next assessed the association of MCID estimates for improvement and worsening of FVC% with patient reported outcomes (PROs) and computer-assisted quantitation of extent of fibrosis (QLF) and of total ILD (QILD) on HRCT.ResultsReliability of FVC%, assessed at a mean of 34 days, was 0.93 for the pooled cohort. The MCID estimates for the pooled cohort at 12 months for FVC% improvement ranged from 3.0 % to 5.3% and for worsening from -3.0% to -3.3%. FVC% improvement by ≥MCID was associated with either statistically significant or numerical improvements in some PROs, QILD, and QLF, while FVC% worsening ≥MCID was associated with statistically significant or numerical worsening of PROs, QILD, and QLF.ConclusionFVC% has acceptable test-retest reliability, and we have provided the MCID estimates for FVC% in SSc-ILD based changes at 12 months from baseline in two clinical trials. Clinical trial registration available at www.clinicaltrials.gov, IDs NCT00004563 and NCT00883129.

Published
2018

5. Open label study of escalating doses of oral treprostinil diethanolamine in patients with systemic sclerosis and digital ischemia: Pharmacokinetics and correlation with digital perfusion

Author

Boin, Francesco, Shah, AA, Schiopu, E, Hummers, LK, Wade, M, Phillips, K, Anderson, C, Wise, R, Seibold, JR, and Wigley, F

Abstract

Introduction: Treprostinil diethanolamine is an innovative salt form of the prostacyclin analogue, treprostinil sodium, developed as an oral sustained release (SR) osmotic tablet. The availability of a formulation permitting convenient systemic delivery mi

Published
2013

6. OxyCAP UK: Oxyfuel Combustion - academic Programme for the UK

Author

Chalmers, H., Al-Jeboori, M., Anthony, B., Balusamy, S., Black, S., Marincola, F. Cavallo, Clements, A., Darabkhani, H., Dennis, J., Farrow, T., Fennell, P., Franchetti, B., Gao, L., Gibbins, J., Hochgreb, S., Hossain, M., Jurado, N., Kempf, A., Liu, H., Lu, G., Ma, L., Navarro-Martinez, S., Nimmo, W., Oakey, J., Pranzitelli, A., Scott, S., Snape, C., Sun, C.-G., Sun, D., Szuhánszki, J., Trabadela, I., Wigley, F., Yan, Y., and Pourkashanian, M.

Published
2014
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9. Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients

Author

Thombs, B.D., Kwakkenbos, L., Carrier, M.E., Bourgeault, A., Tao, L.D., Harb, S., Gagarine, M., Rice, D., Bustamante, L., Ellis, K., Duchek, D., Wu, Y., Bhandari, P.M., Neupane, D., Carboni-Jimenez, A., Henry, R.S., Krishnan, A., Sun, Y., Levis, B., He, C., Turner, K.A., Benedetti, A., Culos-Reed, N., El-Baalbaki, G., Hebblethwaite, S., Bartlett, S.J., Dyas, L., Patten, S., Varga, J., Fortune, C., Gietzen, A., Guillot, G., Lewis, N., Nielsen, K., Richard, M., Sauve, M., Welling, J., Baron, M., Furst, D.E., Gottesman, K., Malcarne, V., Mayes, M.D., Mouthon, L., Nielson, W.R., Riggs, R., Wigley, F., Assassi, S., Boutron, I., Ells, C., Ende, C. van den, Fligelstone, K., Frech, T., Godard, D., Harel, D., Hinchcliff, M., Hudson, M., Johnson, S.R., Larche, M., Leite, C., Nguyen, C., Pope, J., Portales, A., Rannou, F., Reyna, T.S.R., Schouffoer, A.A., Suarez-Almazor, M.E., Agard, C., Albert, A., Andre, M., Arsenault, G., Benzidia, I., Bernstein, E.J., Berthier, S., Bissonnette, L., Boire, G., Bruns, A., Carreira, P., Casadevall, M., Chaigne, B., Chung, L., Cohen, P., Correia, C., Dagenais, P., Denton, C., Domsic, R., Dubois, S., Dunne, J.V., Dunogue, B., Fare, R., Farge-Bancel, D., Fortin, P.R., Gill, A., Gordon, J., Granel-Rey, B., Gyger, G., Hachulla, E., Hatron, P.Y., Herrick, A.L., Hij, A., Hoa, S., Ikic, A., Jones, N., Fernandes, A.J.D., Kafaja, S., Khalidi, N., Lambert, M., Launay, D., Liang, P., Maillard, H., Maltez, N., Manning, J., Marie, I., Martin, M., Martin, T., Masetto, A., Maurier, F., Mekinian, A., Melchor, S., Nikpour, M., Olagne, L., Poindron, V., Proudman, S., Regent, A., Riviere, S., Robinson, D., Rodriguez, E., Roux, S., Smets, P., Smith, D., Sobanski, V., Spiera, R., Steen, V., Stevens, W., Sutton, E., Terrier, B., Thorne, C., Wilcox, P., Ayala, M.C., Ostbo, N., Scleroderma Patient-ctr Interventi, and SPIN Investigators

Subjects
Coronavirus, COVID-19, Systemic sclerosis, Mental health, Anxiety, RCT, Trial, Scleroderma
Abstract

Objective: Contagious disease outbreaks and related restrictions can lead to negative psychological outcomes, particularly in vulnerable populations at risk due to pre-existing medical conditions. No randomised controlled trials (RCTs) have tested interventions to reduce mental health consequences of contagious disease outbreaks. The primary objective of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Trial is to evaluate the effect of a videoconference-based program on symptoms of anxiety. Secondary objectives include evaluating effects on symptoms of depression, stress, loneliness, boredom, physical activity, and social interaction.Methods: The SPIN-CHAT Trial is a pragmatic RCT that will be conducted using the SPIN-COVID-19 Cohort, a sub-cohort of the SPIN Cohort. Eligible participants will be SPIN-COVID-19 Cohort participants without a positive COVID-19 test, with at least mild anxiety (PROMIS Anxiety 4a v1.0 T-score >= 55), not working from home, and not receiving current counselling or psychotherapy. We will randomly assign 162 participants to intervention groups of 7 to 10 participants each or waitlist control. We will use a partially nested RCT design to reflect dependence between individuals in training groups but not in the waitlist control. The SPIN-CHAT Program includes activity engagement, education on strategies to support mental health, and mutual participant support. Intervention participants will receive the 4-week (3 sessions per week) SPIN-CHAT Program via video-conference. The primary outcome is PROMIS Anxiety 4a score immediately post-intervention.Ethics and dissemination: The SPIN-CHAT Trial will test whether a brief videoconference-based intervention will improve mental health outcomes among at-risk individuals during contagious disease outbreak.

Published
2020

10. Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients

Author

Thombs, BD, Kwakkenbos, L, Carrier, M-E, Bourgeault, A, Tao, L, Harb, S, Gagarine, M, Rice, D, Bustamante, L, Ellis, K, Duchek, D, Wu, Y, Bhandari, PM, Neupane, D, Carboni-Jimenez, A, Henry, RS, Krishnan, A, Sun, Y, Levis, B, He, C, Turner, KA, Benedetti, A, Culos-Reed, N, El-Baalbaki, G, Hebblethwaite, S, Bartlett, SJ, Dyas, L, Patten, S, Varga, J, Fortune, C, Gietzen, A, Guillot, G, Lewis, N, Nielsen, K, Richard, M, Sauve, M, Welling, J, Baron, M, Furst, DE, Gottesman, K, Malcarne, V, Mayes, MD, Mouthon, L, Nielson, WR, Riggs, R, Wigley, F, Assassi, S, Boutron, I, Ells, C, van den Ende, C, Fligelstone, K, Frech, T, Godard, D, Harel, D, Hinchcliff, M, Hudson, M, Johnson, SR, Larche, M, Leite, C, Nguyen, C, Pope, J, Portales, A, Rannou, F, Rodriguez Reyna, TS, Schouffoer, AA, Suarez-Almazor, ME, Agard, C, Albert, A, Andre, M, Arsenault, G, Benzidia, I, Bernstein, EJ, Berthier, S, Bissonnette, L, Boire, G, Bruns, A, Carreira, P, Casadevall, M, Chaigne, B, Chung, L, Cohen, P, Correia, C, Dagenais, P, Denton, C, Domsic, R, Dubois, S, Dunne, J, Dunogue, B, Fare, R, Farge-Bancel, D, Fortin, PR, Gill, A, Gordon, J, Granel-Rey, B, Gyger, G, Hachulla, E, Hatron, P-Y, Herrick, AL, Hij, A, Hoa, S, Ikic, A, Jones, N, Fernandes, AJDB, Kafaja, S, Khalidi, N, Lambert, M, Launay, D, Liang, P, Maillard, H, Maltez, N, Manning, J, Marie, I, Martin, M, Martin, T, Masetto, A, Maurier, F, Mekinian, A, Melchor, S, Nikpour, M, Olagne, L, Poindron, V, Proudman, S, Regent, A, Riviere, S, Robinson, D, Rodriguez, E, Roux, S, Smets, P, Smith, D, Sobanski, V, Spiera, R, Steen, V, Stevens, W, Sutton, E, Terrier, B, Thorne, C, Wilcox, P, Ayala, MC, Ostbo, N, Thombs, BD, Kwakkenbos, L, Carrier, M-E, Bourgeault, A, Tao, L, Harb, S, Gagarine, M, Rice, D, Bustamante, L, Ellis, K, Duchek, D, Wu, Y, Bhandari, PM, Neupane, D, Carboni-Jimenez, A, Henry, RS, Krishnan, A, Sun, Y, Levis, B, He, C, Turner, KA, Benedetti, A, Culos-Reed, N, El-Baalbaki, G, Hebblethwaite, S, Bartlett, SJ, Dyas, L, Patten, S, Varga, J, Fortune, C, Gietzen, A, Guillot, G, Lewis, N, Nielsen, K, Richard, M, Sauve, M, Welling, J, Baron, M, Furst, DE, Gottesman, K, Malcarne, V, Mayes, MD, Mouthon, L, Nielson, WR, Riggs, R, Wigley, F, Assassi, S, Boutron, I, Ells, C, van den Ende, C, Fligelstone, K, Frech, T, Godard, D, Harel, D, Hinchcliff, M, Hudson, M, Johnson, SR, Larche, M, Leite, C, Nguyen, C, Pope, J, Portales, A, Rannou, F, Rodriguez Reyna, TS, Schouffoer, AA, Suarez-Almazor, ME, Agard, C, Albert, A, Andre, M, Arsenault, G, Benzidia, I, Bernstein, EJ, Berthier, S, Bissonnette, L, Boire, G, Bruns, A, Carreira, P, Casadevall, M, Chaigne, B, Chung, L, Cohen, P, Correia, C, Dagenais, P, Denton, C, Domsic, R, Dubois, S, Dunne, J, Dunogue, B, Fare, R, Farge-Bancel, D, Fortin, PR, Gill, A, Gordon, J, Granel-Rey, B, Gyger, G, Hachulla, E, Hatron, P-Y, Herrick, AL, Hij, A, Hoa, S, Ikic, A, Jones, N, Fernandes, AJDB, Kafaja, S, Khalidi, N, Lambert, M, Launay, D, Liang, P, Maillard, H, Maltez, N, Manning, J, Marie, I, Martin, M, Martin, T, Masetto, A, Maurier, F, Mekinian, A, Melchor, S, Nikpour, M, Olagne, L, Poindron, V, Proudman, S, Regent, A, Riviere, S, Robinson, D, Rodriguez, E, Roux, S, Smets, P, Smith, D, Sobanski, V, Spiera, R, Steen, V, Stevens, W, Sutton, E, Terrier, B, Thorne, C, Wilcox, P, Ayala, MC, and Ostbo, N

Abstract

Objective Contagious disease outbreaks and related restrictions can lead to negative psychological outcomes, particularly in vulnerable populations at risk due to pre-existing medical conditions. No randomised controlled trials (RCTs) have tested interventions to reduce mental health consequences of contagious disease outbreaks. The primary objective of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Trial is to evaluate the effect of a videoconference-based program on symptoms of anxiety. Secondary objectives include evaluating effects on symptoms of depression, stress, loneliness, boredom, physical activity, and social interaction.

Published
2020

15. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

Author

Kowal-Bielecka, O, Landewé, R, Avouac, J, Chwiesko, S, Miniati, I, Czirjak, L, Clements, P, Denton, C, Farge, D, Fligelstone, K, Földvari, I, Furst, D E, Müller-Ladner, U, Seibold, J, Silver, R M, Takehara, K, Toth, Garay B, Tyndall, A, Valentini, G, van den Hoogen, F, Wigley, F, Zulian, F, and Matucci-Cerinic, Marco

Published
2009
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16. European League Against Rheumatism (EULAR) Scleroderma Trial and Research group (EUSTAR) recommendations for the treatment of systemic sclerosis: methods of elaboration and results of systematic literature research

Author

Avouac, J, Kowal-Bielecka, O, Landewe, R, Chwiesko, S, Miniati, I, Czirjak, L, Clements, P, Denton, C, Farge, D, Fligelstone, K, Földvari, I, Furst, D E, Müller-Ladner, U, Seibold, J, Silver, R M, Takehara, K, Toth, Garay B, Tyndall, A, Valentini, G, van den Hoogen, F, Wigley, F, Zulian, F, and Matucci-Cerinic, M

Published
2009
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20. Shortening patient-reported outcome measures through optimal test assembly: Application to the Social Appearance Anxiety Scale in the Scleroderma Patient-centered Intervention Network Cohort

Author

Harel, D., Mills, S.D., Kwakkenbos, L., Carrier, M.E., Nielsen, K., Portales, A., Bartlett, S.J., Malcarne, V.L., Thombs, B.D., Baron, M., Furst, D.E., Gottesman, K., Mayes, M.D., Mouthon, L., Nielson, W.R., Riggs, R., Sauve, M., Wigley, F., Assassi, S., Boutron, I., Maia, A.C., El-Baalbaki, G., Ells, C., Ende, C. van den, Fligelstone, K., Fortune, C., Frech, T., Godard, D., Hudson, M., Impens, A., Jang, Y., Johnson, S.R., Kennedy, A.T., Korner, A., Larche, M., Leite, C., Marra, C., Pope, J., Reyna, T.S.R., Schouffoer, A.A., Steele, R.J., Suarez-Almazor, M.E., Welling, J., Wong-Rieger, D., Agard, C., Albert, A., Andre, M., Arsenault, G., Benmostefa, N., Benzidia, I., Berthier, S., Bissonnette, L., Boire, G., Bruns, A., Carreira, P., Casadevall, M., Chaigne, B., Chung, L., Cohen, P., Dagenais, P., Denton, C., Domsic, R., Dubois, S., Dunne, J.V., Dunogue, B., Esquinca, A., Fare, R., Farge-Bancel, D., Fortin, P.R., Gill, A., Gordon, J., Granel-Rey, B., Grange, C., Gyger, G., Hachulla, E., Hatron, P.Y., Herrick, A.L., Hij, A., Hinchcliff, M., Ikic, A., Jones, N., Fernandes, A.J.D., Kafaja, S., Khalidi, N., Korman, B., Launay, D., Liang, P., London, J., Luna, D., Maillard, H., Manning, J., Martin, M., Martin, T., Masetto, A., Maurier, F., Mekinian, A., Melchor, S., Nikpour, M., Paule, R., Proudman, S., Regent, A., Riviere, S., Robinson, D., Rodriguez, E., Roux, S., Smets, P., Smith, D., Sobanski, V., Spiera, R., Steen, V., Stevens, W., Sutton, E., Terrier, B., Thorne, C., Varga, J., Wilcox, P., Wilson, M., Cumin, J., Fox, R.S., Gholizadeh, S., Jewett, L.R., Levis, B., Pepin, M.R., Turner, K.A., Lambert, M., and SPIN Investigators

Subjects
Adult, Male, medicine.medical_specialty, systemic sclerosis, Concurrent validity, Anxiety, Fear of negative evaluation, Cohort Studies, Experimental Psychopathology and Treatment, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Cronbach's alpha, medicine, Humans, Patient Reported Outcome Measures, optimal test assembly, 030212 general & internal medicine, Aged, Aged, 80 and over, Scleroderma, Systemic, business.industry, Research, short form, Social anxiety, Reproducibility of Results, generalized partial credit model, General Medicine, Middle Aged, stomatognathic diseases, Cross-Sectional Studies, Convergent validity, patient reported outcome measure, Physical Appearance, Body, Physical therapy, Female, Patient-reported outcome, medicine.symptom, business, 030217 neurology & neurosurgery, Cohort study
Abstract

ObjectivesThe Social Appearance Anxiety Scale (SAAS) is a 16-item measure that assesses social anxiety in situations where appearance is evaluated. The objective was to use optimal test assembly (OTA) methods to develop and validate a short-form SAAS based on objective and reproducible criteria.DesignThis study was a cross-sectional analysis of baseline data from adults enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort.SettingAdults in the SPIN Cohort in the present study were enrolled at 28 centres in Canada, the USA and the UK.ParticipantsThe SAAS was administered to 926 adults with scleroderma.Primary and secondary measuresThe SAAS, Brief Fear of Negative Evaluation II (BFNE II), Brief Satisfaction with Appearance Scale (Brief-SWAP), Patient Health Questionnaire-8 (PHQ8) and Social Interaction Anxiety Scale-6 (SIAS-6) were collected, as well as demographic characteristics.ResultsOTA methods identified a maximally informative shortened version for each possible form length between 1 and 15 items. The final shortened version was selected based on prespecified criteria for reliability, concurrent validity and statistically equivalent convergent validity with the BFNE II scale. A five-item short version was selected (SAAS-5). The SAAS-5 had a Cronbach’s α of 0.95 and had high concurrent validity with the full-length form (r=0.97). The correlation of the SAAS-5 with the BFNE II was 0.66, which was statistically equivalent to that of the full-length form. Furthermore, the correlation of the SAAS-5 with the two subscales of the Brief-SWAP, and the SIAS-6, were statistically equivalent to that of the full-length form.ConclusionsOTA was an efficient method for shortening the full-length SAAS to create the SAAS-5.

Published
2019

25. Update of EULAR recommendations for the treatment of systemic sclerosis

Author

Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., Distler O., Clements P., Cutolo M., Czirjak L., Damjanov N., Del Galdo F., Denton C. P., Distler J. H. W., Foeldvari I., Figelstone K., Frerix M., Furst D. E., Guiducci S., Hunzelmann N., Khanna D., Matucci-Cerinic M., Herrick A. L., Van Den Hoogen F., Van Laar J. M., Riemekasten G., Silver R., Smith V., Sulli A., Tarner I., Tyndall A., Welling J., Wigley F., Valentini G., Walker U. A., Zulian F., Muller-Ladner U., Daikeler T., Lanciano E., Becvar R., Tomcik M., Gindzienska-Sieskiewicz E., Cuomo G., Iudici M., Rednic S., Vlachoyiannopoulos P. G., Caporali R., Carreira P. E., Novak S., Minier T., Kucharz E. J., Gabrielli A., Moroncini G., Airo' P., Hesselstrand R., Martinovic D., Radic M., Marasovic-Krstulovic D., Braun-Moscovici Y., Balbir-Gurman A., Lo Monaco A., Caramaschi P., Morovic-Vergles J., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Seidel M. F., Hasler P., Pereira Da Silva J. A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Ananieva L. P., Beretta L., Szucs G., Szamosi S., de la Puente Bujidos C., Midtvedt O., Hoffmann-Vold A. -M., Launay D., Hachulla E., Riccieri V., Ionescu R., Opris D., Mihai C., Herrgott I., Beyer C., Ingegnoli F., von Muhlen C. A., Alegre-Sancho J. J., Beltran-Catalan E., Aringer M., Fantana J., Leuchten N., Tausche A. -K., De Langhe E., Vanthuyne M., Anic B., Baresic M., Mayer M., Uprus M., Otsa K., Yavuz S., Granel B., Azevedo V. F., Muller C., Jimenez S. A., Popa S., Agachi S., Zenone T., Stebbings S., Dockerty J., Vacca A., Schollum J., Veale D. J., Toloza S., Xu D., Olas J., Rosato E., Foti R., Adler S., Dan D., Wiesik-Szewczyk E., Olesinska M., Kayser C., Fathi N., de la Pena Lefebvre P. G., Imbert B., Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., Clements, P., Cutolo, M., Czirjak, L., Damjanov, N., Del Galdo, F., Denton, C. P., Distler, J. H. W., Foeldvari, I., Figelstone, K., Frerix, M., Furst, D. E., Guiducci, S., Hunzelmann, N., Khanna, D., Matucci-Cerinic, M., Herrick, A. L., Van Den Hoogen, F., Van Laar, J. M., Riemekasten, G., Silver, R., Smith, V., Sulli, A., Tarner, I., Tyndall, A., Welling, J., Wigley, F., Valentini, G., Walker, U. A., Zulian, F., Muller-Ladner, U., Daikeler, T., Lanciano, E., Becvar, R., Tomcik, M., Gindzienska-Sieskiewicz, E., Cuomo, G., Iudici, M., Rednic, S., Vlachoyiannopoulos, P. G., Caporali, R., Carreira, P. E., Novak, S., Minier, T., Kucharz, E. J., Gabrielli, A., Moroncini, G., Airo', P., Hesselstrand, R., Martinovic, D., Radic, M., Marasovic-Krstulovic, D., Braun-Moscovici, Y., Balbir-Gurman, A., Lo Monaco, A., Caramaschi, P., Morovic-Vergles, J., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M. F., Hasler, P., Pereira Da Silva, J. A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Ananieva, L. P., Beretta, L., Szucs, G., Szamosi, S., de la Puente Bujidos, C., Midtvedt, O., Hoffmann-Vold, A. -M., Launay, D., Hachulla, E., Riccieri, V., Ionescu, R., Opris, D., Mihai, C., Herrgott, I., Beyer, C., Ingegnoli, F., von Muhlen, C. A., Alegre-Sancho, J. J., Beltran-Catalan, E., Aringer, M., Fantana, J., Leuchten, N., Tausche, A. -K., De Langhe, E., Vanthuyne, M., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Granel, B., Azevedo, V. F., Muller, C., Jimenez, S. A., Popa, S., Agachi, S., Zenone, T., Stebbings, S., Dockerty, J., Vacca, A., Schollum, J., Veale, D. J., Toloza, S., Xu, D., Olas, J., Rosato, E., Foti, R., Adler, S., Dan, D., Wiesik-Szewczyk, E., Olesinska, M., Kayser, C., Fathi, N., de la Pena Lefebvre, P. G., Imbert, B., UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicola, Khanna, Dinesh, Matucci Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller Ladner, Ulf, Daikeler, Thoma, Lanciano, Elisabetta, Becvã¡r, Radim, Tomcik, Michal, Gindzienska Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tã¼nde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic Krstulovic, Daniela, Braun Moscovici, Yolanda, Monaco, Andrea Lo, Morovic Vergles, Jadranka, Culo, Melanie I., Henes, Jã¶rg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska Jakubus, Malgorzata, Seidel, Matthias F., Klinik III, Medizinische, Hasler, Paul, Da Silva, José A. Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlo, Midtvedt, Øyvind, Hoffmann Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre Sancho, Juan José, Beltran Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Bareå¡ic, Marko, Mayer, Miroslav, Ãœprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma García, Imbert, Bernard, and Cuomo, Giovanna

Subjects
Endothelin Receptor Antagonists, Lung Diseases, Kidney Disease, Delphi Technique, Gastrointestinal Diseases, systemic sclerosis, Scleroderma Renal Crisis, Placebo-controlled study, Angiotensin-Converting Enzyme Inhibitors, Lung Disease, Scleroderma, 0302 clinical medicine, Glucocorticoid, Phosphodiesterase 5 Inhibitor, Immunology and Allergy, skin and connective tissue diseases, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, integumentary system, treatment, genetics and molecular biology (all), Hematopoietic Stem Cell Transplantation, cyclophosphamide, methotrexate, Pulmonary, Orvostudományok, Serotonin Uptake Inhibitor, 3. Good health, Europe, Systematic review, Hypertension, Serotonin Uptake Inhibitors, Cyclophosphamide, Methotrexate, Systemic Sclerosis, Treatment, Fingers, Fluoxetine, Glucocorticoids, Humans, Hypertension, Pulmonary, Kidney Diseases, Phosphodiesterase 5 Inhibitors, Prostaglandins I, Pyrazoles, Pyrimidines, Raynaud Disease, Rheumatology, Scleroderma, Systemic, Ulcer, Immunology, Biochemistry, Genetics and Molecular Biology (all), 030211 gastroenterology & hepatology, Endothelin Receptor Antagonist, Selective Serotonin Reuptake Inhibitors, medicine.drug, Human, medicine.medical_specialty, Gastrointestinal Disease, Klinikai orvostudományok, Riociguat, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, medicine, Finger, biochemistry, Intensive care medicine, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Systemic Sclerosi, 030203 arthritis & rheumatology, business.industry, Systemic, Angiotensin-Converting Enzyme Inhibitor, medicine.disease, Transplantation, Clinical research, Pyrimidine, immunology and allergy, rheumatology, immunology, Pyrazole, Physical therapy, business, Rheumatism
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

Published
2017
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26. 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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30. Progression of Interstitial Lung Disease in Systemic Sclerosis: The Importance of Pneumoproteins Krebs von den Lungen 6 and CCL18

Author

Volkmann, Elizabeth R., primary, Tashkin, Donald P., additional, Kuwana, Masataka, additional, Li, Ning, additional, Roth, Michael D., additional, Charles, Julio, additional, Hant, Faye N., additional, Bogatkevich, Galina S., additional, Akter, Tanjina, additional, Kim, Grace, additional, Goldin, Jonathan, additional, Khanna, Dinesh, additional, Clements, Philip J., additional, Furst, Daniel E., additional, Elashoff, Robert M., additional, Silver, Richard M., additional, Assassi, Shervin, additional, Theodore, A. C., additional, Simms, R. W., additional, Kissin, E., additional, Cheong, F. Y., additional, Steen, V. D., additional, Read, C. A., additional, Fridley, C., additional, Zulmatashvili, M., additional, Wise, R. A., additional, Wigley, F. M., additional, Hummers, L., additional, Leatherman, G., additional, Silver, R. M., additional, Strange, C., additional, Hant, F. N., additional, Ham, J., additional, Gibson, K., additional, Rosson, D., additional, Tashkin, D. P., additional, Elashoff, R. M., additional, Roth, M. D., additional, Clements, P. J., additional, Furst, D., additional, Volkmann, E. R., additional, Kafaja, S., additional, Kleerup, E., additional, Elashoff, D., additional, Goldin, J., additional, Ariola, E., additional, Marlis, G., additional, Mason‐Berry, J., additional, Saffold, P., additional, Rodriguez, M., additional, Guzman, L., additional, Brook, J., additional, Golden, J., additional, Connolly, M. K., additional, Eller, A., additional, Leong, D., additional, Lalosh, M., additional, Obata, J., additional, Volkov, S., additional, Schraufnagel, D., additional, Arami, S., additional, Franklin, D., additional, Varga, J., additional, Dematte, J., additional, Hinchcliff, M., additional, DeLuca, C., additional, Donnelly, H., additional, Marlin, C., additional, Riley, D. J., additional, Hsu, V. M., additional, McCloskey, D. A., additional, Phillips, K., additional, Khanna, D., additional, Martinez, F. J., additional, Schiopu, E., additional, Konkle, J., additional, Mayes, M., additional, Patel, B., additional, Assassi, S., additional, Tan, F., additional, Fischer, A., additional, Swigris, J., additional, Meehan, R., additional, Brown, K., additional, Warren, T., additional, Morrison, M., additional, Scholand, M. B., additional, Frecht, T., additional, Carey, P., additional, Villegas, M., additional, Molitor, J., additional, and Carlson, P., additional

Published
2019
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33. Update of EULAR recommendations for the treatment of systemic sclerosis

Author

Kowal-Bielecka, O. Fransen, J. Avouac, J. Becker, M. Kulak, A. Allanore, Y. Distler, O. Clements, P. Cutolo, M. Czirjak, L. Damjanov, N. Del Galdo, F. Denton, C.P. Distler, J.H.W. Foeldvari, I. Figelstone, K. Frerix, M. Furst, D.E. Guiducci, S. Hunzelmann, N. Khanna, D. Matucci-Cerinic, M. Herrick, A.L. Van Den Hoogen, F. Van Laar, J.M. Riemekasten, G. Silver, R. Smith, V. Sulli, A. Tarner, I. Tyndall, A. Welling, J. Wigley, F. Valentini, G. Walker, U.A. Zulian, F. Müller-Ladner, U. EUSTAR Coauthors Daikeler, T. Lanciano, E. Becvár, R. Tomcik, M. Gińdzieńska-Sieskiewicz, E. Cuomo, G. Iudici, M. Rednic, S. Vlachoyiannopoulos, P.G. Caporali, R. Carreira, P.E. Novak, S. Minier, T. Kucharz, E.J. Gabrielli, A. Moroncini, G. Airo', P. Hesselstrand, R. Martinovic, D. Radic, M. Marasovic-Krstulovic, D. Braun-Moscovici, Y. Balbir-Gurman, A. Lo Monaco, A. Caramaschi, P. Morovic-Vergles, J. Henes, J. Ortiz Santamaria, V. Heitmann, S. Krasowska, D. Seidel, M.F. Hasler, P. Pereira Da Silva, J.A. Salvador, M.J. Stamenkovic, B. Stankovic, A. Tikly, M. Ananieva, L.P. Beretta, L. Szucs, G. Szamosi, S. de la Puente Bujidos, C. Midtvedt, Ø. Hoffmann-Vold, A.-M. Launay, D. Hachulla, E. Riccieri, V. Ionescu, R. Opris, D. Mihai, C. Herrgott, I. Beyer, C. Ingegnoli, F. von Mühlen, C.A. Alegre-Sancho, J.J. Beltrán-Catalán, E. Aringer, M. Fantana, J. Leuchten, N. Tausche, A.-K. De Langhe, E. Vanthuyne, M. Anic, B. Barešic, M. Mayer, M. Üprus, M. Otsa, K. Yavuz, S. Granel, B. Azevedo, V.F. Muller, C. Jimenez, S.A. Popa, S. Agachi, S. Zenone, T. Stebbings, S. Dockerty, J. Vacca, A. Schollum, J. Veale, D.J. Toloza, S. Xu, D. Olas, J. Rosato, E. Foti, R. Adler, S. Dan, D. Wiesik-Szewczyk, E. Olesińska, M. Kayser, C. Fathi, N. de la Peña Lefebvre, P.G. Imbert, B.

Subjects
integumentary system, skin and connective tissue diseases
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc. © Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Published
2017

35. Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study

Author

Jaeger, VK, Wirz, EG, Allanore, Y, Rossbach, P, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, PE, Balbir Gurman, A, Tikly, M, Vettori, S, Damjanov, N, Müller-Ladner, U, Distler, JHW, Li, M, Walker, UA, EUSTAR co-authors, Ananieva, L, Heitmann, S, Rednic, S, Riccieri, V, Szmyrka-Kaczmarek, M, Farge, D, Lapadula, G, Matucci-Cerinic, M, Guiducci, S, Hunzelmann, N, Ricci, M, Mihai, C, Veale, D, Hesselstrand, R, Mariok, E, Smith, V, Tarner, IH, Kucharz, EJ, Czirjak, L, Martinovic, D, Solanki, K, Ancuta, CM, Sibilia, J, Paola, C, Hassanien, M, Kahl, S, Wigley, F, Vanthuyne, M, Opris, D, Radominski, SC, Lo Monaco, A, Corrado, A, Koehm, M, Codullo, V, Radim, B, Loyo, E, Uprus, M, Pellerito, R, Zenone, T, Gabrielli, A, Kowal-Bielecka, O, Rozman, B, Scorza, R, Saketkoo, LA, Midtvedt, O, von Muhlen, A, Henes, J, Branimir, A, Hasler, P, Yavuz, S, Adler, S, Krummel-Lorenz, B, Posa, M, Engelhart, M, Denton, C, Krasowska, D, Garcia de la Pena Lefebvre, P, Cozzi, F, Mouthon, L, Rosato, E, Selmi, C, Sancho, JJA, Mallia, C, Limonta, M, Seidel, M, Foti, R, Stamp, L, Ullman, S, Stebbings, S, Santamaria, VO, Del Galdo, F, De Langhe, E, Mathieu, A, Sunderkotter, C, Eyerich, K, Stamenkovic, B, Novak, S, Sampaio-Barros, PD, Kayser, C, Litinsky, I, Couto, M, and Assassi, S

Subjects
integumentary system
Abstract

Objective Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. Methods In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. Results Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. Conclusion In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to ‘widen' the still very narrow ‘window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.

Published
2016

36. European League Against Rheumatism (EULAR) Scleroderma Trial and Research group (EUSTAR) recommendations for the treatment of systemic sclerosis: methods of elaboration and results of systematic literature research

Author

Avouac J., Kowal-Bielecka O., Landewe R., Chwiesko S., Miniati I., Czirjak L., Clements P., Denton C., Farge D., Fligelstone K., Foldvari I., Furst D. E., Muller-Ladner U., Seibold J., Silver R. M., Takehara K., Garay Toth B., Tyndall A., Valentini Gabriele., Van Den Hoogen F., Wigley F., Zulian F., Matucci-Cerinic M., and EUSTAR coauthors, Cuomo Giovanna, et al, Avouac, J, Kowal Bielecka, O, Landewe, R, Chwiesko, S, Miniati, I, Czirjak, L, Clements, P, Denton, C, Farge, D, Fligelstone, K, Földvari, I, Furst, De, Müller Ladner, U, Seibold, J, Silver, Rm, Takehara, K, Toth, Bg, Tyndall, A, Valentini, Gabriele, van den Hoogen, F, Wigley, F, Zulian, F, Matucci Cerinic, M, Eustar, Coauthor, Cuomo, Giovanna, Avouac, J., Kowal-Bielecka, O., Landewe, R., Chwiesko, S., Miniati, I., Czirjak, L., Clements, P., Denton, C., Farge, D., Fligelstone, K., Foldvari, I., Furst, D. E., Muller-Ladner, U., Seibold, J., Silver, R. M., Takehara, K., Garay Toth, B., Tyndall, A., Valentini, Gabriele., Van Den Hoogen, F., Wigley, F., Zulian, F., Matucci-Cerinic, M., and EUSTAR, Coauthor, and Et, Al

Subjects
Research design, Pathology, medicine.medical_specialty, Evidence-based practice, Consensus Development Conferences as Topic, Immunology, Delphi method, MEDLINE, General Biochemistry, Genetics and Molecular Biology, Rheumatology, medicine, Humans, Immunology and Allergy, Randomized Controlled Trials as Topic, Evidence-Based Medicine, Scleroderma, Systemic, business.industry, Evidence-based medicine, Number needed to harm, Jadad scale, Review Literature as Topic, Treatment Outcome, Research Design, Family medicine, Number needed to treat, business, Human
Abstract

Objective: To describe methods and procedures used for the development of the European League Against Rheumatism (EULAR)/EULAR Scleroderma Trial and Research group (EUSTAR) recommendations for the treatment of systemic sclerosis. In particular, the results of a web-based Delphi exercise aimed at selection of research questions and evidence from systematic literature research, as parts of the development of these recommendations, are presented in detail. Methods: In agreement with the EULAR standard operating procedures a Task Force was created that consisted of the EUSTAR board members, 10 systemic sclerosis (SSc) experts invited from outside the EUSTAR board and representing Europe, the USA and Japan, a clinical epidemiologist, 2 patients with SSc and 3 fellows for literature research. All EUSTAR centres were invited to contribute to the development of recommendations through submission and preliminary selection of the research questions. The systematic literature research was performed using the Pubmed, Medline, EMBASE and Cochrane databases. Retrieved trials were evaluated according to the Jadad classification, and the level of evidence was graded from 1 to 4. Outcome data for efficacy and adverse events were abstracted and effect size, number needed to treat (NNT) and number needed to harm (NNH) were calculated when appropriate. Results: In all, 65 EUSTAR Centres provided 304 research questions concerning SSc treatment. These questions were aggregated, subdivided into 19 treatment categories and then subjected to preliminary selection by a web-based Delphi technique. The final set of 26 research questions was created by the Expert Committee based on the results of the Delphi exercise and the expert’s experience. Conclusions: This paper is a comprehensive summary of the methods we used to build recommendations for the drug treatment of systemic sclerosis, combining an evidence based approach and expert opinion. Objective: To describe methods and procedures used for the development of the European League Against Rheumatism (EULAR)/EULAR Scleroderma Trial and Research group (EUSTAR) recommendations for the treatment of systemic sclerosis. In particular, the results of a web-based Delphi exercise aimed at selection of research questions and evidence from systematic literature research, as parts of the development of these recommendations, are presented in detail. Methods: In agreement with the EULAR standard operating procedures a Task Force was created that consisted of the EUSTAR board members, 10 systemic sclerosis (SSc) experts invited from outside the EUSTAR board and representing Europe, the USA and Japan, a clinical epidemiologist, 2 patients with SSc and 3 fellows for literature research. All EUSTAR centres were invited to contribute to the development of recommendations through submission and preliminary selection of the research questions. The systematic literature research was performed using the Pubmed, Medline, EMBASE and Cochrane databases. Retrieved trials were evaluated according to the Jadad classification, and the level of evidence was graded from 1 to 4. Outcome data for efficacy and adverse events were abstracted and effect size, number needed to treat (NNT) and number needed to harm (NNH) were calculated when appropriate. Results: In all, 65 EUSTAR Centres provided 304 research questions concerning SSc treatment. These questions were aggregated, subdivided into 19 treatment categories and then subjected to preliminary selection by a web-based Delphi technique. The final set of 26 research questions was created by the Expert Committee based on the results of the Delphi exercise and the expert's experience. Conclusions: This paper is a comprehensive summary of the methods we used to build recommendations for the drug treatment of systemic sclerosis, combining an evidence based approach and expert opinion.

Published
2009

37. Defining appropriate outcome measures in pulmonary arterial hypertension related to systemic sclerosis: A Delphi consensus study with cluster analysis

Author

Distler, O, Behrens, F, Pittrow, D, Huscher, D, Denton, Cp, Foeldvari, I, Humbert, M, Matucci Cerinic, M, Nash, P, Opitz, Cf, Rubin, Lj, Seibold, Jr, Furst, De, EPOSS Omeract Group including Ahmadi Simab, K, Albera, Carlo, Bolster, Mb, Brühlmann, P, Burger, C, Chan, K, Chatterjee, S, Clements, P, Confalonieri, M, Csuka, Me, Farber, H, Fessler, B, Foley, R, Frantz, R, Gran, Jt, Highland, K, Hoeper, M, Hsu, V, Inanc, M, Jansa, P, Johnson, S, Kahaleh, B, Kawut, Sm, Keogh, A, Khanna, D, Kähler, Cm, Lang, I, Mahmud, Th, Mandel, J, Mathier, M, Mayes, M, Mchugh, N, Mckown, K, Mclaughlin, V, Medsger TA Jr, Mehta, S, Merkel, Pa, Mubarak, K, Nathan, S, Oudiz, R, Palevsky, H, Park, M, Pope, J, Presberg, K, Ralph, D, Rich, S, Rothfield, N, Rubenfire, M, Scorza, R, Senecal, Jl, Shanahan, J, Silver, R, Staehler, G, Steen, V, Strange, C, Sweiss, N, Taichman, D, Talwar, A, Voskuyl, A, Wigley, F, Williamson, T, Wollheim, F., and University of Zurich

Subjects
Cardiac Catheterization, medicine.medical_specialty, Delphi Technique, Visual analogue scale, Hypertension, Pulmonary, 2745 Rheumatology, Immunology, Delphi method, Placebo-controlled study, Blood Pressure, 610 Medicine & health, Severity of Illness Index, law.invention, Rheumatology, Quality of life, Randomized controlled trial, law, Outcome Assessment, Health Care, Severity of illness, Cluster Analysis, Immunology and Allergy, Medicine, Pharmacology (medical), Randomized Controlled Trials as Topic, Scleroderma, Systemic, business.industry, 10051 Rheumatology Clinic and Institute of Physical Medicine, Clinical trial, Blood pressure, Echocardiography, Exercise Test, Quality of Life, Physical therapy, business
Abstract

Objective. Outcome measures for pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc) are only partially validated. The aim of the present study was to establish an expert consensus regarding which outcome measures are most appropriate for clinical trials in PAH-SSc. Methods. Sixty-nine PAH-SSc experts (rheumatologists, cardiologists, pulmonologists) rated a list of disease domains and measurement tools in an Internet-based 3-stage Delphi consensus study. In stages 2 and 3, the medians of domains and measurement tools and frequency distributions of ratings, along with requests for re-ratings, were distributed to respondents to provide feedback. A final score of items was identified by means of cluster analysis. Results. The experts judged the following domains and tools as most appropriate for randomized controlled trials in PAH-SSc: lung vascular/pulmonary arterial pressure and cardiac function both measured by right heart catheterization and echocardiography, exercise testing measured by 6-minute walking test and oxygen saturation at exercise, severity of dyspnea measured on a visual analog scale, discontinuation of treatment measured by (serious) adverse events, quality of life/activities of daily living measured by the Short Form 36 and Health Assessment Questionnaire disability index, and global state assessed by physician measured by survival. Conclusion. Among experts in PAH-SSc, a core set of outcome measures has been defined for clinical trials by Delphi consensus methods. Although these outcome measures are recommended by this expert group to be used as an interim tool, it will be necessary to formally validate the present measures, as well as potential research measures, in further studies.

Published
2008
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38. Biomarkers of fibrosis

Author

Varga, J, Denton, C P, Wigley, F M, Varga, J ( J ), Denton, C P ( C P ), Wigley, F M ( F M ), Beyer, C, Distler, O, Distler, J H W, Varga, J, Denton, C P, Wigley, F M, Varga, J ( J ), Denton, C P ( C P ), Wigley, F M ( F M ), Beyer, C, Distler, O, and Distler, J H W

Abstract

Biomarkers are modern diagnostics that can guide the treatment of our patients in daily clinical life and direct drug development in preclinical and clinical studies. Biological markers allow screening for diseases, giving prognostic information, and assessing disease activity. Over the last 20 years, biomarkers helped to prioritize drug development and minimize risks for drug attrition in late-phase clinical studies. Despite their central roles in modern medicine, the numbers of disease-specific biomarkers that we routinely assess in our patients with systemic sclerosis (SSc) are limited. So far, past medical history, clinical examination, and equipment-based diagnostics (e.g., computed tomography, body plethysmography, and right-heart catheterization studies) dominate our treatment decisions in SSc.

Published
2012

39. 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
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40. 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

41. Development of a provisional core set of response measures for clinical trials of systemic sclerosis

Author

Khanna D, Lovell DJ, Giannini E, Clements PJ, Merkel PA, Seibold JR, Matucci Cerinic M, Denton CP, Mayes MD, Steen VD, Varga J, Furst DE, Baron M, Csuka ME, Berezne A, Briet SN, Brühlmann P, Buch MH, Catoggio L, Collier D, Crofford L, Czirják L, Derk CT, Distler O, Doyle MK, Farge Bancel D, Fessler B, Foeldvari I, Goldberg A, Gran JT, Grau R, Griffing WL, Hayat S, Herrick AL, Hsu V, Hummers LK, Inanç M, Johnson S, Kahaleh MB, Lafyatis RA, Lee P, Mahmud TH, Malcarne V, McHugh NJ, Martin RW, McKown K, Medsger TA Jr, Moreland L, Pope JE, Rich E, Rothfield NF, Schiopu E, Scorza R, Senécal JL, Shanahan J, Simms RW, Strand V, Silver RM, Sweiss N, van den Hoogen FH, Veale D, Voskuyl AE, Wigley F, Wollheim FA, VALENTINI, Gabriele, Khanna, D, Lovell, Dj, Giannini, E, Clements, Pj, Merkel, Pa, Seibold, Jr, Matucci Cerinic, M, Denton, Cp, Mayes, Md, Steen, Vd, Varga, J, Furst, De, Baron, M, Csuka, Me, Berezne, A, Briet, Sn, Brühlmann, P, Buch, Mh, Catoggio, L, Collier, D, Crofford, L, Czirják, L, Derk, Ct, Distler, O, Doyle, Mk, Farge Bancel, D, Fessler, B, Foeldvari, I, Goldberg, A, Gran, Jt, Grau, R, Griffing, Wl, Hayat, S, Herrick, Al, Hsu, V, Hummers, Lk, Inanç, M, Johnson, S, Kahaleh, Mb, Lafyatis, Ra, Lee, P, Mahmud, Th, Malcarne, V, Mchugh, Nj, Martin, Rw, Mckown, K, Medsger TA, Jr, Moreland, L, Pope, Je, Rich, E, Rothfield, Nf, Schiopu, E, Scorza, R, Senécal, Jl, Shanahan, J, Simms, Rw, Strand, V, Silver, Rm, Sweiss, N, Valentini, Gabriele, van den Hoogen, Fh, Veale, D, Voskuyl, Ae, Wigley, F, and Wollheim, Fa

Subjects
medicine.medical_specialty, Consensus, Delphi Technique, Visual analogue scale, Endpoint Determination, Immunology, Alternative medicine, Delphi method, General Biochemistry, Genetics and Molecular Biology, Article, Rheumatology, Epidemiology, Immunology and Allergy, Medicine, Humans, Multicenter Studies as Topic, computer.programming_language, Core set, Clinical Trials as Topic, Scleroderma, Systemic, business.industry, Outcome measures, Clinical trial, Treatment Outcome, Physical therapy, business, computer, Delphi
Abstract

Objective: To develop a provisional core set of response measures for clinical trials of systemic sclerosis (SSc). Methods: The Scleroderma Clinical Trials Consortium (SCTC) conducted a structured, 3-round Delphi exercise to reach consensus on a core set of measures for clinical trials of SSc. Round 1 asked the SCTC investigators to list items in 11 pre-defined domains (skin, musculoskeletal, cardiac, pulmonary, cardio-pulmonary, gastrointestinal, renal, Raynaud phenomenon and digital ulcers, health-related quality of life and function, global health, and biomarkers) for SSc clinical trials. Round 2 asked respondents to rate the importance of the chosen items and was followed by a meeting, during which the Steering Committee discussed the feasibility, reliability, redundancy and validity of the items. Round 3 sought to obtain broader consensus on the core set measures. Members also voted on items that had data on feasibility but lacked data on reliability and validity, but may still be useful research outcome measures for future trials. Results: A total of 50 SCTC investigators participated in round 1, providing 212 unique items for the 11 domains. In all, 46 (92%) participants responded in round 2 and rated 177 items. The ratings of 177 items were reviewed by the Steering Committee and 31 items from the 11 domains were judged to be appropriate for inclusion in a 1-year multi-centre clinical trial. In total, 40 SCTC investigators completed round 3 and ranked 30 of 31 items as acceptable for inclusion in the core set. The Steering Committee also proposed 14 items for a research agenda. Conclusion: Using a Delphi exercise, we have developed a provisional core set of measures for assessment of disease activity and severity in clinical trials of SSc.

Published
2008

42. Characteristics of joint involvement and relationship with systemic inflammation in systemic sclerosis: result from the EULAR scleroderma trial and research (EUSTAR) database

Author

Avouac, J, Walker, U, Tyndall, A, Kahan, A, Matucci Cerinic, M, Allanore, Y, Eustar, Miniati, I, Muller, A, Iannone, F, Distler, O, Becvar, R, Sierakowsky, S, Kowal Bielecka, O, Coelho, P, Cabane, J, Cutolo, M, Shoenfeld, Y, Valentini, G, Rovensky, J, Riemekasten, G, Vlachoyiannopoulos, P, Caporali, R, Jiri, S, Inanc, M, Zimmermann Gorska, I, Carreira, P, Novak, S, Czirjak, L, Oliveira Ramos, F, Jendro, M, Chizzolini, C, Kucharz, Ej, Richter, J, Cozzi, F, Rozman, B, Mallia, Cm, Gabrielli, A, Farge, D, Kiener, Hp, Schöffel, D, Airo, P, Wollheim, F, Martinovic, D, Trotta, F, Jablonska, S, Reich, K, Bombardieri, S, Siakka, P, Pellerito, R, Bambara, Lm, 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 JM, Kaltwasser, Jp, Foeldvari, I, Juan Mas, A, Bajocchi, G, Wislowska, M, Pereira Da Silva JA, Jacobsen, S, Worm, M, Graniger, W, Kuhn, A, Stankovic, A, Cossutta, R, Majdan, M, Damjanovska Rajcevska, L, Tikly, M, Nasonov, El, Steinbrink, K, Herrick, A, Müller Ladner, U, Dinc, A, Scorza, R, Sondergaard, K, Indiveri, F, Nielsen, H, Szekanecz, Z, Silver, Rm, Antivalle, M, Espinosa, Ib, García de la Pena Lefebvre, P, Midtvedt, O, Launay, D, Valesini, F, Tuvik, P, Ionescu, Rm, Del Papa, N, Pinto, S, Wigley, F, Mihai, C, Sinziana Capranu, M, Sunderkötter, C, Jun, Jb, Alhasani, S, Distler, Jh, Ton, E, Soukup, T, Seibold, J, Zeni, S, Nash, P, Mouthon, L, De Keyser, F, Duruöz, Mt, Cantatore, Fp, Strauss, G, von Mülhen CA, Pozzi, Mr, Eyerich, K, Szechinski, J, Keiserman, M, Houssiau, Fa, Román Ivorra JA, Krummel Lorenz, B, Aringer, M, Westhovens, R, Bellisai, F, Mayer, M, Stoeckl, F, Uprus, M, Volpe, A, Buslau, M, Yavuz, S, Granel, B, Valderílio Feijó, A, Del Galdo, F, Popa, S, Zenone, T, Ricardo Machado, X, Pileckyte, M, Stebbings, S, Mathieu, A, Tulli, A, Tourinho, T, Souza, R, Acayaba de Toledo, R, Stamp, L, Solanki, K, Veale, D, Francisco Marques Neto, J, Bagnato, Gf, Loyo, E, Toloza, S, Li, M, Ahmed Abdel Atty Mohamed, W, Cobankara, V, Olas, J, Salsano, F, Oksel, F, Tanaseanu, Cm, Foti, R, Ancuta, C, Vonk, M, Caramaschi, Paola, Beretta, L, Balbir, A, Chiàla, A, Pasalic Simic, K, Ghio, M, Stamenkovic, B, Rednic, S, Host, N, Hachulla, E, and Furst, D. E.

Subjects
joint involvement, Systemic sclerosis, synovitis
Published
2010

43. 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, J., Walker, U., Tyndall, A., Kahan, A., Matucci-Cerinic, M., Allanore, Y., 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, G., Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I.Z., Carreira, P., Novak, S., Czirjak, L., Ramos, F.O., 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.D., 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.S., 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.V., Del Galdo, F., Popa, S., Zenone, T., Machado, X.R., Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J.F.M., Bagnato, G.F., Loyo, E., Toloza, S., Li, M., Mohamed, W.A.A.A., Çobankara, Veli, 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.P., Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., and Furst, D.E.

Subjects
musculoskeletal diseases, Adult, Male, Databases, Factual, systemic sclerosis, joint contracture, hand radiography, Joint involvement, Tendons, Scleroderma, Localized, data base, joint radiography, Humans, scleroderma, human, nuclear magnetic resonance imaging, Range of Motion, Articular, rheumatic disease, Aged, interstitial lung disease, Inflammation, skin disease, Clinical Trials as Topic, Synovitis, Scleroderma, Systemic, integumentary system, article, echography, Middle Aged, musculoskeletal system, cohort analysis, major clinical study, tenosynovitis, clinical feature, body regions, female, Cross-Sectional Studies, priority journal, Tendon friction rub, Joints, disease duration, Joint Diseases, 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 amore severe disease and with systemic inflammation. The Journal of Rheumatology Copyright © 2010. All rights reserved.

Published
2010

46. Efficacy of Mycophenolate Mofetil and Oral Cyclophosphamide on Skin Thickness: Post Hoc Analyses From Two Randomized Placebo‐Controlled Trials

Author

Namas, Rajaie, Tashkin, Donald P., Furst, Daniel E., Wilhalme, Holly, Tseng, Chi‐Hong, Roth, Michael D., Kafaja, Suzanne, Volkmann, Elizabeth, Clements, Philip J., Khanna, Dinesh, Elashoff, R., Goldin, J., Roth, M., Furst, D., Bulpitt, K., Chung, W.‐L. J., Viasco, S., Sterz, M., Woolcock, L., Yan, X., Ho, J., Vasunilashorn, S., Costa, I., Seibold, J. R., Riley, D. J., Amorosa, J. K., Hsu, V. M., McCloskey, D. A., Wilson, J. E., Varga, J., Schraufnagel, D., Wilbur, A., Lapota, D., Arami, S., Cole‐Saffold, P., Simms, R., Theodore, A., Clarke, P., Korn, J., Tobin, K., Nuite, M., Silver, R., Bolster, M., Strange, C., Schabel, S., Smith, E., Arnold, J., Caldwell, K., Bonner, M., Wise, R., Wigley, F., White, B., Hummers, L., Bohlman, M., Polito, A., Leatherman, G., Forbes, E., Daniel, M., Martin, D., Steen, V., Read, C., Cooper, C., Wheaton, S., Carey, A., Ortiz, A., Mayes, M., Parsley, E., Oldham, S., Filemon, T., Jordan, S., Perry, M., Connolly, K., Golden, J., Wolters, P., Webb, R., Davis, J., Antolos, C., Maynetto, C., Fessler, B., Olman, M., Sanders, C., Heck, L., Parkhill, T., Rothfield, N., Metersky, M., Cobb, R., Aberles, M., Ingenito, F., Breen, E., Mayes, M., Mubarak, K., Granda, J. L., Silva, J., Injic, Z., Alexander, R., Furst, D., Springmeyer, S., Kirkland, S., Molitor, J., Hinke, R., Mondt, A., Thompson, T., Rounds, S., Weinstein, M., Thompson, B., Paulus, H., Levy, S., Kissin, E., Cheong, F.Y., Marlis, G., Mason‐Berry, J., Saffold, P., Rodriguez, M., Guzman, L., Brook, J., Ibrahim, G., Largaespada, K., Fridley, C., Zulmastashvili, M., Manu, A., Moore, S., Hummers, L., Leatherman, G., Hant, F. N., Gibson, K., Morrison, M., Donnelly, H., Marlin, C., Gangar, J., McCloskey, D. A., Eller, A., Leong, D., Lalosh, M., Obata, J., Arami, S., Franklin, D., Schiopu, E., Benedict‐Blue, M., Leone, V., Shaw, J., Tan, F., Perry, M., Anderson, J., Saulino, A., Carey, P., Esplin, M., and Carlson, P.

Abstract

To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on modified Rodnan skin score (MRSS) in participants enrolled in the Scleroderma Lung Study (SLS) I and II. SLSI participants received daily oral CYCor matching placebo for 1 year, whereas SLS IIparticipants received daily MMFfor 2 years or daily oral CYCfor 1 year followed by placebo for second year. We assessed the impact of MMFand CYCon the MRSSin SLS IIover a 24‐month period. We also compared the change in MRSSin patients with diffuse cutaneous systemic sclerosis (dcSSc) assigned to CYCand MMFin SLS IIand SLSI versus placebo in SLSI over a 24‐month period using a linear mixed model. In SLS II, the baseline mean ± SD MRSSwas 14.0 ± 10.6 units for CYCand 15.3 ± 10.4 units for MMF; 58.5% were classified as dcSSc. CYCand MMFwere associated with statistically significant improvements in MRSSfrom baseline over the period of 24 months in dcSSc (P< 0.05 at each time point), but there were no differences between the 2 groups. In the dcSSc subgroup, the change in MRSSfrom baseline to all 6‐month visits was similar in SLS IIgroups (MMF,CYC, pooled cohort [MMF+ CYC]) and in the SLSI CYCgroup and showed statistically significant improvements compared to SLSI placebo at 12, 18, and 24 months (P< 0.05). In SLS II,MMFand CYCtreatment resulted in improvements in MRSSin patients with dcSSc over 24 months. In addition, MMFand CYCtreatment resulted in statistically significant improvements in MRSSin patients with dcSSc when compared with the SLSI placebo group.

Published
2018
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47. A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS)

Author

Stone, J, Hoffman, G, Merkel, P, Min, Y, Uhlfelder, M, Hellmann, D, Specks, U, Allen, N, Davis, J, Spiera, R, Calabrese, L, Wigley, F, Maiden, N, Valente, R, Niles, J, Fye, K, McCune, J, St Clair, E, and Luqmani, R

Abstract

OBJECTIVE: To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease-specific activity index for Wegener's granulomatosis (WG). METHODS: Sixteen members of the International Network for the Study of the Systemic Vasculitides (INSSYS) revised the BVAS, with 3 goals: to reduce the redundancy of some component items, to enhance its ability to capture important disease manifestations specific to WG, and to streamline the instrument for use in clinical research. We defined the items and weighted them empirically as either minor (e.g., nasal crusting = 1 point) or major (e.g., alveolar hemorrhage = 3 points). We then validated the new, disease-specific BVAS/WG in 2 simulation exercises and a clinical case series that involved 117 patients with WG. RESULTS: We removed 38 items from the original BVAS, revised 9 items, and added 7 new items. Correlations between the scores on the BVAS/WG and the physician's global assessment (PGA) of disease activity were high, even when patients in remission were excluded. In the clinical case series, Spearman's rank correlation coefficient between the BVAS/WG and the PGA was r = 0.81 (95% confidence interval 0.73-0.87). The interobserver reliability using intraclass (within-case) correlation coefficients in the 2 simulation exercises was r = 0.93 for the BVAS/WG and r = 0.88 for the PGA in the first and r = 0.91 for the BVAS/WG and r = 0.88 for the PGA in the second. There was no significant observer effect in the scoring of the BVAS/WG or the PGA. The discriminant validity of the BVAS/WG was good: r = 0.73 (95% confidence interval 0.43-0.83). CONCLUSION: The BVAS/WG is a valid, disease-specific activity index for WG. Tested in simulation exercises and in actual patients, the BVAS/WG correlates well with the PGA, is sensitive to change, and has good inter- and intraobserver reliability. The INSSYS will use the BVAS/WG to assess the primary outcome in a phase II/III trial of etanercept in WG.

Published
2001

48. Mechanisms for accumulation and migration of technetium-99 in saltmarsh sediments

Author

Wigley, F.

Abstract

This thesis describes the development of analytical methods for both the bulk determination of 99Tc, and determination of 99Tc in sequential extracts from sediments. These methods have been used to collect data, which, along with trace and major element data have been used to interpret the mechanisms for 99Tc input, migration and accumulation in saltmarshes. The inventory of 99Tc stored in the Thornflatt Saltmarsh, Esk Estuary has also been determined. The routine determination of 99Tc in bulk samples uses 99mTc as a yield monitor. Samples are ignited stepwise to 550°C and the 99Tc is extracted using 8M nitric acid. Many contaminants are precipitated with Fe(OH)3 and the Tc in the supernant is pre-concentrated and further purified using anion-exchange chromatography. Final separation of Tc from Ru is achieved by extraction of Tc into 5% TnOA in xylene from 2M sulphuric acid. The yield is determined by γ-spectrometric analysis of 99mTc. Determination of 99Tc is made by liquid scintillation counting. Typical recoveries are in the order of 70-95% and the method has a detection limit of 1.7 Bq/kg for a sample size of 10g. Determination of Tc in sequential extracts uses operationally defined procedures to extract: exchangeable Tc, reducible Tc and oxidisable Tc. An initial water wash is used to extract any occluded Tc and a final leach in 8 M nitric acid is used to dissolve any residual Tc. The isolation of 99Tc uses TEVA resin for Extracts 1-4 and the decontamination procedure developed for bulk analysis for Extract 5. 99mTc was used as a yield monitor, and determination of 99Tc is by liquid scintillation counting. Limits of detection were dependent on the amount of 99mTc tracer used but were found to be as low as 2.4 Bq/kg for a sample size of 2g. A study was made of the mechanisms responsible for the accumulation and migration of Tc in estuarine sediments using sediments collected from saltmarshes at Thornflatt, Carlaverock and the Ribble Estuary. 99Tc was present at determinable activities in all the sediment cores taken from these sites. Good correlations between Tc and CaO as well as CO3 concentrations and poor correlation between Tc and radionuclides adsorbed to inorganic detritus infer a direct input of 99Tc to marsh sediments. Determination of 99Tc in biota living on the marsh also showed that this was not a significant pathway for input of Tc to the sediments. Sequential extraction data imply sorption to an organic fraction of the sediment. Stable element and sequential extraction data indicates that Tc is readily oxidised and remobilised before reprecipitation where redox conditions are favourable. Data indicate a reduction potential between those of the MnIV to MnII reaction and the FeIII to FeII reaction is necessary for re-accumulation to occur, as suggested by published thermodynamic data. Data collected from reducing sediments imply that similar mechanisms are responsible for the accumulation of Mn (e.g. reduction by sulphate reducing bacteria) and the accumulation of Tc. The inventory of 99Tc held within the Thornflatt saltmarsh is proportionally less than that of 137Cs or 241Am when compared to discharges from Sellafield. However a higher proportion of 99Tc is transferred from Seliafield and incorporated into saltmarsh sediments than is suggested by previously published standard distribution coefficient data. Saltmarsh sediments are therefore a more important sink of 99Tc than extrapolations made from inventories of other radionuclides would suggest.

Published
2000

49. Identification of novel genetic markers associated with clinical phenotypes of systemic sclerosis through a genome-wide association strategy.

Author

Gorlova, Olga, Martín, J. E., Rueda, B., Koeleman, B. P., Ying, J., Díaz-Gallo, L. M., Broen, Jasper C., Vonk, Madelon C., Simeón, Carmen P., Alizadeh, B. Z., Coenen, M. J., Voskuyl, Alexandre E., Schuerwegh, A. J., Riel, Piet L.C.M. van, Vanthuyne, M., van 't Slot, Rubén, Italiaander, A., Ophoff, Roel A., Hunzelmann, Nicolas, Fonollosa, V., Ortego-Centeno, N., González-Gay, M. A., García-Hernández, Francisco José, González-Escribano, María Francisca, Airó, Paolo, Laar, Jacob M. van, Worthington, J., Hesselstrand, R., Smith, V., Keyser, F. de, Houssiau, F., Chee, M. M., Madhok, R., Shiels, Paul G., Westhovens, R., Kreuter, A., Baere, E. de, Witte, Torsten, Padyukov, Leonid, Nordin, A., Scorza, R., Lunardi, C., Lie, B. A., Hoffmann-Vold, A. M., Palm, Øyvind, García de la Peña, P., Carreira, P., Spanish Scleroderma Group, Varga, J., Hinchcliff, M., Lee, Annette T., Gourh, P., Amos, C. I., Wigley, F. M., Hummers, L. K., Hummers, J., Nelson, J. L., Riemekasten, G., Herrick, A., Beretta, L., Fonseca, C., Denton, C., Gregersen, Peter K., Agarwal, S. K., Assassi, S., Tan, Filemon K., Arnett, F. C., Radstake, T. R., Mayes, Maureen D., Martín, J., Teruel, María, Gorlova, Olga, Martín, J. E., Rueda, B., Koeleman, B. P., Ying, J., Díaz-Gallo, L. M., Broen, Jasper C., Vonk, Madelon C., Simeón, Carmen P., Alizadeh, B. Z., Coenen, M. J., Voskuyl, Alexandre E., Schuerwegh, A. J., Riel, Piet L.C.M. van, Vanthuyne, M., van 't Slot, Rubén, Italiaander, A., Ophoff, Roel A., Hunzelmann, Nicolas, Fonollosa, V., Ortego-Centeno, N., González-Gay, M. A., García-Hernández, Francisco José, González-Escribano, María Francisca, Airó, Paolo, Laar, Jacob M. van, Worthington, J., Hesselstrand, R., Smith, V., Keyser, F. de, Houssiau, F., Chee, M. M., Madhok, R., Shiels, Paul G., Westhovens, R., Kreuter, A., Baere, E. de, Witte, Torsten, Padyukov, Leonid, Nordin, A., Scorza, R., Lunardi, C., Lie, B. A., Hoffmann-Vold, A. M., Palm, Øyvind, García de la Peña, P., Carreira, P., Spanish Scleroderma Group, Varga, J., Hinchcliff, M., Lee, Annette T., Gourh, P., Amos, C. I., Wigley, F. M., Hummers, L. K., Hummers, J., Nelson, J. L., Riemekasten, G., Herrick, A., Beretta, L., Fonseca, C., Denton, C., Gregersen, Peter K., Agarwal, S. K., Assassi, S., Tan, Filemon K., Arnett, F. C., Radstake, T. R., Mayes, Maureen D., Martín, J., and Teruel, María

Abstract

The aim of this study was to determine, through a genome-wide association study (GWAS), the genetic components contributing to different clinical sub-phenotypes of systemic sclerosis (SSc).We considered limited (lcSSc) and diffuse (dcSSc) cutaneous involvement, and the relationships with presence of the SSc-specific auto-antibodies, anti-centromere (ACA), and anti-topoisomerase I (ATA). Four GWAS cohorts, comprising 2,296 SSc patients and 5,171 healthy controls, were meta-analyzed looking for associations in the selected subgroups. Eighteen polymorphisms were further tested in nine independent cohorts comprising an additional 3,175 SSc patients and 4,971 controls. Conditional analysis for associated SNPs in the HLA region was performed to explore their independent association in antibody subgroups. Overall analysis showed that non-HLA polymorphism rs11642873 in IRF8 gene to be associated at GWAS level with lcSSc (P = 2.32×10−12, OR = 0.75). Also, rs12540874 in GRB10 gene (P = 1.27 × 10−6, OR = 1.15) and rs11047102 in SOX5 gene (P = 1.39×10−7, OR = 1.36) showed a suggestive association with lcSSc and ACA subgroups respectively. In the HLA region, we observed highly associated allelic combinations in the HLA-DQB1 locus with ACA (P = 1.79×10−61, OR = 2.48), in the HLA-DPA1/B1 loci with ATA (P = 4.57×10−76, OR = 8.84), and in NOTCH4 with ACA P = 8.84×10−21, OR = 0.55) and ATA (P = 1.14×10−8, OR = 0.54). We have identified three new non-HLA genes (IRF8, GRB10, and SOX5) associated with SSc clinical and auto-antibody subgroups. Within the HLA region, HLA-DQB1, HLA-DPA1/B1, and NOTCH4 associations with SSc are likely confined to specific auto-antibodies. These data emphasize the differential genetic components of subphenotypes of SSc

Published
2011

50. Impacts of fuel quality on power production

Author

Harding, S., Wall, T., Wigley, F., Frandsen, Flemming, Hupa, Mikko, Tillman, D., Harding, S., Wall, T., Wigley, F., Frandsen, Flemming, Hupa, Mikko, and Tillman, D.

Abstract

The first ash deposition or slagging and fouling conference, as they are commonly called, was held at the Marchwood Engineering Laboratories in the UK in 1963. Since that time many excellent conferences have occurred to provide interchange, dialogue, experiences and results among those who deal with the many opportunities afforded by fuel quality. However, due to the changing sponsorship environment, it has been several years since the last conference which focused on fuel characteristics and their paramount importance in power production. This conference, the 17th in the series, was entitled “Impacts of Fuel Quality on Power Production” and was held in Snowbird, UT, from October 29 to November 3, 2006. More than 50 participants from 13 countries throughout the world participated.

Published
2007

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