Update of EULAR recommendations for the treatment of systemic sclerosis

Research output: Contribution to journalArticle


title = "Update of EULAR recommendations for the treatment of systemic sclerosis",
abstract = "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.",
author = "Otylia Kowal-Bielecka and Jaap Fransen and Jerome Avouac and Mike Becker and Agnieszka Kulak and Yannick Allanore and Oliver Distler and Philip Clements and Maurizio Cutolo and Laszlo Czirjak and Nemanja Damjanov and {del Galdo}, Francesco and Denton, {Christopher P.} and Distler, {J{\"o}rg H W} and Ivan Foeldvari and Kim Figelstone and Marc Frerix and Furst, {Daniel E.} and Serena Guiducci and Nicolas Hunzelmann and Dinesh Khanna and Marco Matucci-Cerinic and Herrick, {Ariane L.} and {van den Hoogen}, Frank and {van Laar}, {Jacob M.} and Gabriela Riemekasten and Richard Silver and Vanessa Smith and Alberto Sulli and Ingo Tarner and Alan Tyndall and Joep Welling and Frederic Wigley and Gabriele Valentini and Walker, {Ulrich A.} and Francesco Zulian and Ulf M{\"u}ller-Ladner and Thomas Daikeler and Elisabetta Lanciano and Radim Becv{\'a}r and Michal Tomcik and Ewa Gindzienska-Sieskiewicz and Michele Iudici and Simona Rednic and Vlachoyiannopoulos, {Panayiotis G.} and Roberto Caporali and Carreira, {Patricia E.} and Srdan Novak and T{\"u}nde Minier and Kucharz, {Eugene J.} and Armando Gabrielli and Gianluca Moroncini and Paolo Airo and Roger Hesselstrand and Duska Martinovic and Mislav Radic and Daniela Marasovic-Krstulovic and Yolanda Braun-Moscovici and Monaco, {Andrea Lo} and Jadranka Morovic-Vergles and Culo, {Melanie I.} and J{\"o}rg Henes and Santamaria, {Vera Ortiz} and Stefan Heitmann and Dorota Krasowska and Malgorzata Michalska-Jakubus and Seidel, {Matthias F.} and Medizinische Klinik and Paul Hasler and {Da Silva}, {Jos{\'e} A Pereira} and Salvador, {Maria J.} and Bojana Stamenkovic and Aleksandra Stankovic and Mohammed Tikly and Ananieva, {Lidia P.} and Lorenzo Beretta and Gabriella Szucs and Szilvia Szamosi and {de la Puente Bujidos}, Carlos and {\O}yvind Midtvedt and Hoffmann-Vold, {Anna Maria} and David Launay and Eric Hachulla and Valeria Riccieri and Ruxandra Ionescu and Daniela Opris and Carina Mihai and Ilka Herrgott and Christian Beyer and Francesca Ingegnoli and {von M{\"u}hlen}, {Carlos Alberto} and Alegre-Sancho, {Juan Jos{\'e}} and Emma Beltran-Catalan and Martin Aringer and Julia Fantana and Nicolai Leuchten and Tausche, {Anne Kathrin} and Langhe, {Ellen De} and Marie Vanthuyne and Branimir Anic and Marko Bare{\v s}ic and Miroslav Mayer and Maria {\"u}prus and Kati Otsa and Sule Yavuz and Brigitte Granel and Jimenez, {Sergio A.} and Serghei Popa and Svetlana Agachi and Thierry Zenone and Simon Stebbings and Joanne Dockerty and Alessandra Vacca and Joanna Schollum and Veale, {Douglas J.} and Sergio Toloza and Dong Xu and Jacek Olas and Edoardo Rosato and Rosario Foti and Sabine Adler and Diana Dan and Ewa Wiesik-Szewczyk and Marzena Olesinska and Cristiane Kayser and Nihal Fathi and {de la Pe{\~n}a Lefebvre}, {Paloma Garc{\'i}a} and Bernard Imbert",
year = "2017",
doi = "10.1136/annrheumdis-2016-209909",
language = "English",
volume = "76",
pages = "1327--1339",
journal = "Annals of the Rheumatic Diseases",
issn = "1468-2060",
publisher = "BMJ Publishing Group",