Article Text

EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)
  1. O Kowal-Bielecka1,
  2. R Landewé2,
  3. J Avouac3,
  4. S Chwiesko1,
  5. I Miniati4,
  6. L Czirjak5,
  7. P Clements6,
  8. C Denton7,
  9. D Farge8,
  10. K Fligelstone9,
  11. I Földvari10,
  12. D E Furst6,
  13. U Müller-Ladner11,
  14. J Seibold12,
  15. R M Silver13,
  16. K Takehara14,
  17. B Garay Toth15,
  18. A Tyndall16,
  19. G Valentini17,
  20. F van den Hoogen18,
  21. F Wigley19,
  22. F Zulian20,
  23. Marco Matucci-Cerinic4,
  24. and the EUSTAR co-authors
  1. 1 Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
  2. 2 Department of Internal Medicine/Rheumatology, University Hospital Maastricht, Maastricht, The Netherlands
  3. 3 Rheumatology A Department, RDU, Paris, France
  4. 4 Department BioMedicine, Division of Rheumatology, University of Florence, Florence, Italy
  5. 5 Rheumatology Department, UP, Pecs, Hungary
  6. 6 Rheumatology Department, UCLA, Los Angeles, California, USA
  7. 7 Rheumatology Department, RFH, London, UK
  8. 8 Internal Medicine Department, HSL, Paris, France
  9. 9 Scleroderma Association, FESCA, Harlesden, UK
  10. 10 Department of Pediatric Rheumatology, Klin Eilbek, Hamburg, Germany
  11. 11 Rheumatology Department, JLU, Bad Nauheim, Germany
  12. 12 Rheumatology Department, UMI, Ann Arbor, Michigan, USA
  13. 13 Rheumatology Department, MUSC, Charleston, Sorth Carolina, USA
  14. 14 Dermatology Department, USM, Kanazawa, Japan
  15. 15 Scleroderma Association, FESCA, Budapest, Hungary
  16. 16 Rheumatology Department, UH, Basel, Switzerland
  17. 17 Rheumatology Department, SU, Naples, Italy
  18. 18 Rheumatology Centre Sint Maartenskliniek, Nijmegen, The Netherlands
  19. 19 Rheumatology Department, JHU, Baltimore, Maryland, USA
  20. 20 Department of Pediatrics, University of Padua, Padua, Italy
  1. Dr M Matucci-Cerinic, Department of BioMedicine and Surgery, Division of Rheumatology, University of Florence, Villa Monna Tessa, viale G Pieraccini 18, 50134 Florence, Italy; cerinic{at}


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.

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Supplementary materials


  • Competing interests: None.

  • Funding: This study received financial support from the European League Against Rheumatism.

  • ▸ Additional supplementary material and tables 3 and 4 are published online only at

  • *EUSTAR co-authors: Lund (F Wollheim, A Scheja); Moskwa (T Nevskaya, L Ananieva, E Nassonov); Paris (Y Allanore, A Kahan); Lille (E Hachulla, D Launay); Manchester (A Herrick); Newcastle (J van Laar); Geneva (C Chizzolini); Bucharest (C Ciurtin, D Opris, R Ionescu, M Capraru); Cluj-Napoca (S Rednic); Zurich (O Distler); Ankara (I Simsek, A Dinc); Istanbul (M Inanc); Graz (W Graninger); Tubingen (I Kotter); Oslo (JT Gran, O Midvedt); Katowice (E Kucharz); Lublin (D Krasowska, M Majdan); Bialystok (S Sierakowski); Coimbra (MJ Salvador, JA Pereira da Silva); Belgrade (N Damjanov); Madrid (P Carreira, P Garcia de la Pena Lefebvre); Debrecen (G Szucs, ZSzekanecz); Praha (R Becvar); Porto (P Pinto); Lisbon (P Coelho); Rijeka (S Novak); Basle (U Walker); Dresden (M Aringer); Gottingen (T Glaenz, CH Neumann, S Emmert); Berlin (M Worm, G Riemekasten); Koln (T Krieg, N Hunzelmann); Mainz (K Steinbrink); Muenster (C Sunderkotter); Stuttgart (S Heitmann); Hamburg (I Foldvari); Bari (F Iannone, G Lapadula); Brescia (P Airň); L’Aquila (R Giacomelli); Milan (R Scorza); Genova (M Rizzi, F Indiveri); Rome (V Riccieri); Padova (F Zulian); Ljubljana (B Rozman); Tokyo (K Takehara); Seoul (Jae-Bum Jun); Philadalphia, (C Derk, N Sandorfi, S Jimenez); Johannesburg (M Tikly); Mosul (Saad Alhasani); Tel Hashomer (Y Shoenfeld); Haifa (A Balbir Gurmann).