Update of EULAR recommendations for the treatment of systemic sclerosis

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 Foeldvari, Kim Figelstone, M. 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 Welling, F 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 Daikeler, Elisabetta Lanciano, Radim Bečvář, Michal Tomcik, Ewa Gińdzieńska-Sieśkiewicz, Giovanna Cuomo, Michele Iudici, Simona Rednic, Panayiotis G. Vlachoyiannopoulos, Roberto Caporali, Patricia E. Carreira, Servicio de Reumatología, Srdan Novak, Tünde Minier, Eugene J. Kucharz, Armando Gabrielli, Gianluca Moroncini, Paolo Airo', Roger Hesselstrand, Duska Martinovic, Mislav Radić, Daniela Marasovic-Krstulovic, Yolanda Braun-Moscovici, Alexandra Balbir-Gurman, Andrea Lo Monaco, Paola Caramaschi, Jadranka Morović-Vergles, Melanie I. Čulo, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Małgorzata Michalska-Jakubus, Matthias F. Seidel, Paul Hasler, Klinik für Rheumatologie, José A. Pereira Da Silva, Maria J. Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Mohammed Tikly, Lidia P. Ananieva, Lorenzo Beretta, Gabriella Szucs, Szilvia Szamosi, Carlos de la Puente Bujidos, Servicio de Reumatología, Øyvind Midtvedt, Anna-Maria Hoffmann-Vold, David Launay, Eric Hachulla, Valeria Riccieri, Ruxandra Ionescu, Daniela Opris, Carina Mihai, Ilka Herrgott, Christian Beyer, Francesca Ingegnoli, Carlos Alberto von Mühlen, Juan José Alegre-Sancho, Emma Beltrán-Catalán, Martin Aringer, Julia Fantana, Nicolai Leuchten, Anne-Kathrin Tausche, Ellen De Langhe, Marie Vanthuyne, Branimir Anic, Marko Barešić, Miroslav Mayer, Maria Üprus, Kati Otsa, Sule Yavuz, Brigitte Granel, Valderilio F. Azevedo, Carolina Muller, Sergio A Jimenez, Serghei Popa, Svetlana Agachi, Thierry Zenone, Simon Stebbings, Joanne Dockerty, Alessandra Vacca, Joanna Schollum, Douglas J. Veale, Sergio Toloza, Dong Xu, Jacek Olas, Edoardo Rosato, Rosario Foti, Sabine Adler, Diana Dan, Ewa Wiesik-Szewczyk, Marzena Olesińska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Bernard Imbert
Annals of the Rheumatic Diseases
November 10, 2016
10.1136/annrheumdis-2016-209909
Cited by 1,113Open Access
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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.

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