TY - JOUR T1 - Update of EULAR recommendations for the treatment of systemic sclerosis JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 1327 LP - 1339 DO - 10.1136/annrheumdis-2016-209909 VL - 76 IS - 8 AU - Otylia Kowal-Bielecka AU - Jaap Fransen AU - Jerome Avouac AU - Mike Becker AU - Agnieszka Kulak AU - Yannick Allanore AU - Oliver Distler AU - Philip Clements AU - Maurizio Cutolo AU - Laszlo Czirjak AU - Nemanja Damjanov AU - Francesco del Galdo AU - Christopher P Denton AU - Jörg H W Distler AU - Ivan Foeldvari AU - Kim Figelstone AU - Marc Frerix AU - Daniel E Furst AU - Serena Guiducci AU - Nicolas Hunzelmann AU - Dinesh Khanna AU - Marco Matucci-Cerinic AU - Ariane L Herrick AU - Frank van den Hoogen AU - Jacob M van Laar AU - Gabriela Riemekasten AU - Richard Silver AU - Vanessa Smith AU - Alberto Sulli AU - Ingo Tarner AU - Alan Tyndall AU - Joep Welling AU - Frederic Wigley AU - Gabriele Valentini AU - Ulrich A Walker AU - Francesco Zulian AU - Ulf Müller-Ladner A2 - , Y1 - 2017/08/01 UR - http://ard.bmj.com/content/76/8/1327.abstract N2 - 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. ER -