ObjectivesThe aim was to update the 2009 EULAR recommendations for the treatment of systemic sclerosis (SSc) [1] with a distinct focus on new therapeutic aspects.MethodsRevision and update of the previous recommendations were performed according to the EULAR standard operating procedures. The task force consisted of 30 SSc experts from Europe and USA, two patients nominated by the pan-European patients association FESCA, a clinical epidemiologist and 3 fellows for systematic literature research. All centers from the EULAR Scleroderma Trials and Research (EUSTAR) group were invited to submit and select research questions concerning SSc treatment using a Delphi approach. A set of 46 research questions addressing 26 different interventions was selected for systematic literature research. The new recommendations were developed in a meeting, based on the available evidence while using a consensus procedure.ResultsSixteen recommendations were developed (instead of 14 in 2009) which address treatments 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 2015 recommendations include phosphodiestase-5 (PDE5) inhibitors in the treatment of SSc-related RP and DUs, riociguat and new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE5 inhibitors for SSc-related PAH. The new recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressing SSc were added. In addition, the statement regarding sitaxentan for PAH was removed, because it was withdrawn from the market. A web-based internal evaluation of the new recommendations revealed high level of approval among task force members (average score >7 out of maximum 9) for all statements except the one regarding fluoxetine for RP (average score of 6,1). In addition, several comments regarding other treatments addressed in research questions and suggestions for the future SSc research agenda were formulated by the experts.ConclusionsThese updated and improved, data- and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations will also facilitate the directions for future clinical research in SSc.ReferencesKowal-Bielecka et al. Ann Rheum Dis 2009;68:620)AcknowledgementsThe project is funded by a research grant of EULAR to the EUSTAR SSc recommendation group. All contributing experts will be listed as full coauthors on the respective presentations and publications.Disclosure of InterestO. Kowal-Bielecka Speakers bureau: Abbvie, Actelion, Pfizer, Roche, J. Fransen: None declared, J. Avouac: None declared, M. Becker Consultant for: Actelion, A. Kulak: None declared, Y. Allanore Consultant for: Bayer Pharma, Actelion, Pfizer, Sanofi-Aventis, CSL Behring, Roche, O. Distler Consultant for: 4D Sc...
Background Joint involvement is frequent and strongly contributes to impaired quality of life in systemic sclerosis (SSc). In a previous cross-sectional study, synovitis and tendon friction rubs were associated with systemic inflammation and disease activity. Objectives Therefore, the aim of the present study was to determine whether synovitis and tendon friction rubs may predict disease progression and severity. Methods We included patients from the EUSTAR database (MEDS online) with early disease duration (first non Raynaud’s symptom equal or less than 3 years) and with a follow-up of at least two years. We extracted data regarding the presence or not of synovitis (tender and swelling joints) and tendon friction rubs (rubbing sensation detected as the tendon was moved) and data related to disease progression. Skin progression was defined by a ≥10% worsening of the modified Rodnan skin score (mRSS). Pulmonary progression was defined by the new onset of pulmonary fibrosis on high resolution CT scan, or the deterioration of lung volume (≥10% of forced vital capacity, FVC). Cardio-vascular worsening was defined for skin by new ischemic digital ulcers (DU), for lung by pre-capillary pulmonary arterial hypertension (PAH) on right heart catheterization, and for heart by the reduction of the left ventricular ejection fraction below 50%. Renal progression was defined by the occurrence of scleroderma renal crisis. Results From the 9165 patients included in the database, 1301 patients (1079 females) met our inclusion criteria (mean ± SD age of 55±15 years, mean ± SD follow-up: 4.5±2.2 years). Synovitis was identified as a predictor of skin progression (Log-rank test, p=0.008). Synovitis remained predictive of skin progression after stratification for disease subset and autoantibody status (Hazard Ratio, HR: 2.39, 95% confidence interval, CI: 1.4-4.98). No impact on lung outcomes was identified. Synovitis predicted vascular progression both for the occurrence of new ischemic DU in the whole cohort of SSc patients (p=0.005), and in the subset of patients without history of DU at baseline (p=0.005), such as development of PAH (p=0.03) and left ventricular dysfunction (p=0.003). Tendon friction rubs predicted skin progression (p=0.02), PAH (p=0.001) and scleroderma renal crisis (p=0.003). Conclusions This first report of the prospective follow-up of EUSTAR patients identified for the first time the merit of baseline synovitis and extended previous data for tendon friction rubs in early SSc patients. These results obtained through the largest worldwide database support the use of these easily detected clinical findings for the risk stratification of SSc patients. These parameters might be used in the future to select high-risk patients, guide therapies and might be regarded as potential surrogate markers for severity. Disclosure of Interest: None Declared
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