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

O. Kowal-Bielecka, R. Landewé, J. Avouac, S. Chwiesko, I. Miniati, L. Czirjak, P. Clements, C. Denton, D. Farge, K. Fligelstone, I. Földvari, D. E. Furst, U. Müller-Ladner, J. Seibold, R. M. Silver, K. Takehara, B. Garay Toth, A. Tyndall, G. Valentini, F. Van Den HoogenF. Wigley, F. Zulian, Marco Matucci-Cerinic, F. Wollheim, A. Scheja, T. Nevskaya, L. Ananieva, E. Nassonov, Y. Allanore, A. Kahan, E. Hachulla, D. Launay, A. Herrick, J. Van Laar, C. Chizzolini, C. Ciurtin, D. Opris, R. Ionescu, M. Capraru, S. Rednic, O. Distler, I. Simsek, A. Dinc, M. Inanc, W. Graninger, I. Kotter, J. T. Gran, O. Midvedt, E. Kucharz, D. Krasowska, M. Majdan, S. Sierakowski, M. J. Salvador, J. A. Pereira Da Silva, N. Damjanov, P. Carreira, P. Garcia De La Pena Lefebvre, G. Szucs, Z. Szekanecz, R. Becvar, P. Pinto, P. Coelho, S. Novak, U. Walker, M. Aringer, T. Glaenz, C. H. Neumann, S. Emmert, M. Worm, G. Riemekasten, T. Krieg, N. Hunzelmann, K. Steinbrink, C. Sunderkotter, S. Heitmann, I. Foldvari, F. Iannone, G. Lapadula, P. Airň, R. Giacomelli, R. Scorza, M. Rizzi, F. Indiveri, V. Riccieri, F. Zulian, B. Rozman, K. Takehara, Jae Bum Jun, C. Derk, N. Sandorfi, S. Jimenez, M. Tikly, Saad Alhasani, Y. Shoenfeld, A. Balbir Gurmann

Research output: Contribution to journalArticle

463 Citations (Scopus)

Abstract

Purpose: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. Methods: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. Results: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. Conclusions: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

Original languageEnglish
Pages (from-to)620-628
Number of pages9
JournalAnnals of the rheumatic diseases
Volume68
Issue number5
DOIs
Publication statusPublished - May 2009

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

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    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., Garay Toth, B., Tyndall, A., Valentini, G., ... Balbir Gurmann, A. (2009). EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Annals of the rheumatic diseases, 68(5), 620-628. https://doi.org/10.1136/ard.2008.096677