RT Journal Article SR Electronic T1 Update of EULAR recommendations for the treatment of systemic sclerosis JF Annals of the Rheumatic Diseases JO Ann Rheum Dis FD BMJ Publishing Group Ltd and European League Against Rheumatism SP 1327 OP 1339 DO 10.1136/annrheumdis-2016-209909 VO 76 IS 8 A1 Otylia Kowal-Bielecka A1 Jaap Fransen A1 Jerome Avouac A1 Mike Becker A1 Agnieszka Kulak A1 Yannick Allanore A1 Oliver Distler A1 Philip Clements A1 Maurizio Cutolo A1 Laszlo Czirjak A1 Nemanja Damjanov A1 Francesco del Galdo A1 Christopher P Denton A1 Jörg H W Distler A1 Ivan Foeldvari A1 Kim Figelstone A1 Marc Frerix A1 Daniel E Furst A1 Serena Guiducci A1 Nicolas Hunzelmann A1 Dinesh Khanna A1 Marco Matucci-Cerinic A1 Ariane L Herrick A1 Frank van den Hoogen A1 Jacob M van Laar A1 Gabriela Riemekasten A1 Richard Silver A1 Vanessa Smith A1 Alberto Sulli A1 Ingo Tarner A1 Alan Tyndall A1 Joep Welling A1 Frederic Wigley A1 Gabriele Valentini A1 Ulrich A Walker A1 Francesco Zulian A1 Ulf Müller-Ladner A1 , YR 2017 UL http://ard.bmj.com/content/76/8/1327.abstract AB The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment 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 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.