Otylia Kowal‐Bielecka✉(Medical University of Białystok)Jaap Fransen(Radboud University Nijmegen)Jérôme Avouac(Délégation Paris 5)Mike Oliver Becker(University Hospital of Zurich)Agnieszka Kulak(Medical University of Białystok)Yannick Allanore(Délégation Paris 5)Oliver Distler(University Hospital of Zurich)Philip J. Clements(University of California, Los Angeles)Maurizio Cutolo(Ospedale Policlinico San Martino)László Czirják(University of Pecs)Nemanja Damjanov(University of Belgrade)Francesco Del Galdo(University of Leeds)Christopher P. Denton(University College London)Jörg H. W. Distler(Friedrich-Alexander-Universität Erlangen-Nürnberg)Ivan FoeldvariKim FigelstoneM. Frerix(University of Hildesheim)Daniel E Furst(University of California, Los Angeles)Serena Guiducci(University of Florence)Nicolas Hunzelmann(University of Cologne)Dinesh Khanna(University of Michigan)Marco Matucci‐Cerinic(University of Florence)Ariane L. Herrick(Manchester Academic Health Science Centre)F.H.J. van den Hoogen(Radboud University Nijmegen)Jacob M. van Laar(University Medical Center Utrecht)Gabriela Riemekasten(University of Lübeck)Richard M. Silver(Medical University of South Carolina)Vanessa Smith(Ghent University Hospital)Alberto Sulli(Ospedale Policlinico San Martino)Ingo H. Tarner(University of Hildesheim)Alan Tyndall(University of Basel)Joep WellingF Wigley(Johns Hopkins University)Gabriele Valentini(University of Campania "Luigi Vanvitelli")Ulrich A. Walker(University of Basel)Francesco Zulian(University of Padua)Ulf Müller‐Ladner(University of Hildesheim)Thomas DaikelerElisabetta LancianoRadim BečvářMichal TomcikEwa Gińdzieńska-SieśkiewiczGiovanna CuomoMichele IudiciSimona RednicPanayiotis G. VlachoyiannopoulosRoberto CaporaliPatricia E. CarreiraServicio de ReumatologíaSrdan NovakTünde MinierEugene J. KucharzArmando GabrielliGianluca MoronciniPaolo Airo'Roger HesselstrandDuska MartinovicMislav RadićDaniela Marasovic-KrstulovicYolanda Braun-MoscoviciAlexandra Balbir-GurmanAndrea Lo MonacoPaola CaramaschiJadranka Morović-VerglesMelanie I. ČuloJörg HenesVera Ortiz SantamariaStefan HeitmannDorota KrasowskaMałgorzata Michalska-JakubusMatthias F. SeidelPaul HaslerKlinik für RheumatologieJosé A. Pereira Da SilvaMaria J. SalvadorBojana StamenkovicAleksandra StankovicMohammed TiklyLidia P. AnanievaLorenzo BerettaGabriella SzucsSzilvia SzamosiCarlos de la Puente BujidosServicio de ReumatologíaØyvind MidtvedtAnna-Maria Hoffmann-VoldDavid LaunayEric HachullaValeria RiccieriRuxandra IonescuDaniela OprisCarina MihaiIlka HerrgottChristian BeyerFrancesca IngegnoliCarlos Alberto von MühlenJuan José Alegre-SanchoEmma Beltrán-CatalánMartin AringerJulia FantanaNicolai LeuchtenAnne-Kathrin TauscheEllen De LangheMarie VanthuyneBranimir AnicMarko BarešićMiroslav MayerMaria ÜprusKati OtsaSule YavuzBrigitte GranelValderilio F. AzevedoCarolina MullerSergio A JimenezSerghei PopaSvetlana AgachiThierry ZenoneSimon StebbingsJoanne DockertyAlessandra VaccaJoanna SchollumDouglas J. VealeSergio TolozaDong XuJacek OlasEdoardo RosatoRosario FotiSabine AdlerDiana DanEwa Wiesik-SzewczykMarzena OlesińskaCristiane KayserNihal FathiPaloma García de la Peña LefebvreBernard Imbert
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.
