Purpose: 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 EUSTAR (EULAR Scleroderma Trials and Research) group 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, United States and Japan, 2 SSc patients and 3 fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centers.
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: 3 on SSc-related digital vasculopathy (Raynaud’s phenomenon and ulcers), 4 on SSc-related pulmonary arterial hypertension, 3 on SSc-related gastrointestinal involvement, 2 on scleroderma renal crisis, 1 on SSc-related interstitial lung disease and 1 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.