Presentation: EULAR/EUSTAR Recommendations for the Treatment of Systemic Sclerosis (SSc) (2007)

30 EULAR/EUSTAR Recommendations for the Treatment of Systemic Sclerosis (SSc)

PURPOSE: Optimal treatment of systemic sclerosis (SSc) is a challenge because SSc is a rare disease with many outcomes affecting several organ systems. The aim of the EUSTAR (EULAR Scleroderma Trials and Research) group was to develop evidence-based 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 (Dougados 2004). 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. After a process of aggregation and data reduction by a Delphi technique, these questions were presented to experts who selected the final set of 26 questions for the systematic literature research performed using Pubmed, Medline, EMBASE, and Cochrane databases. Retrieved trials were evaluated according to Jadad classification (Jadad 1996), and the level of evidence was graded from Ia to IV, using a previously reported classification (Shekelle 1999). Outcome data for efficacy and adverse events were abstracted and effect size, NNT and NNH calculated when appropriate.
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 SS-related interstitial lung disease and 1 on skin involvement. Experts also formulated several questions for a future research agenda.
CONCLUSIONS: This first systematic approach to recommendations for SSc treatment developed useful recommendations for clinicians treating patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

 M. Matucci-Cerinic, None.