Update of EULAR recommendations for the treatment of systemic sclerosis

Research output: Contribution to journalArticle

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.

Details

Authors
  • Otylia Kowal-Bielecka
  • Jaap Fransen
  • Jerome Avouac
  • Mike Becker
  • Agnieszka Kulak
  • Yannick Allanore
  • Oliver Distler
  • Philip Clements
  • Maurizio Cutolo
  • Laszlo Czirjak
  • Nemanja Damjanov
  • Francesco del Galdo
  • Christopher P. Denton
  • Jörg H W Distler
  • Ivan Foeldvari
  • Kim Figelstone
  • Marc Frerix
  • Daniel E. Furst
  • Serena Guiducci
  • Nicolas Hunzelmann
  • Dinesh Khanna
  • Marco Matucci-Cerinic
  • Ariane L. Herrick
  • Frank van den Hoogen
  • Jacob M. van Laar
  • Gabriela Riemekasten
  • Richard Silver
  • Vanessa Smith
  • Alberto Sulli
  • Ingo Tarner
  • Alan Tyndall
  • Joep Welling
  • Frederic Wigley
  • Gabriele Valentini
  • Ulrich A. Walker
  • Francesco Zulian
  • Ulf Müller-Ladner
  • Thomas Daikeler
  • Elisabetta Lanciano
  • Radim Becvár
  • Michal Tomcik
  • Ewa Gindzienska-Sieskiewicz
  • Michele Iudici
  • Simona Rednic
  • Panayiotis G. Vlachoyiannopoulos
  • Roberto Caporali
  • Patricia E. Carreira
  • Srdan Novak
  • Tünde Minier
  • Eugene J. Kucharz
  • Armando Gabrielli
  • Gianluca Moroncini
  • Paolo Airo
  • Duska Martinovic
  • Mislav Radic
  • Daniela Marasovic-Krstulovic
  • Yolanda Braun-Moscovici
  • Andrea Lo Monaco
  • Jadranka Morovic-Vergles
  • Melanie I. Culo
  • Jörg Henes
  • Vera Ortiz Santamaria
  • Stefan Heitmann
  • Dorota Krasowska
  • Malgorzata Michalska-Jakubus
  • Matthias F. Seidel
  • Medizinische Klinik
  • Paul Hasler
  • 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
  • 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 Beltran-Catalan
  • Martin Aringer
  • Julia Fantana
  • Nicolai Leuchten
  • Anne Kathrin Tausche
  • Ellen De Langhe
  • Marie Vanthuyne
  • Branimir Anic
  • Marko Barešic
  • Miroslav Mayer
  • Maria üprus
  • Kati Otsa
  • Sule Yavuz
  • Brigitte Granel
  • 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 Olesinska
  • Cristiane Kayser
  • Nihal Fathi
  • Paloma García de la Peña Lefebvre
  • Bernard Imbert
Organisations
External organisations
  • Medical University of Bialystok
  • Radboud Institute for Health Sciences
  • Paris Descartes University
  • University Hospital of Zurich
  • University of California, Los Angeles
  • Ospedale Policlinico San Martino
  • University of Pécs
  • University of Belgrade
  • University of Leeds
  • Royal Free Hospital
  • Friedrich-Alexander University Erlangen-Nürnberg
  • Hamburg Center for Pediatric and Adolescent Rheumatology
  • Federation of European Scleroderma Associations aisbl (FESCA)
  • Justus Liebig University Giessen
  • University of Florence
  • University of Cologne
  • University of Michigan
  • Great Ormond Street Hospital
  • University Medical Center Utrecht
  • British Columbia Children's Hospital
  • Medical University of South Carolina
  • Ghent University Hospital
  • University of Basel
  • FESCA Patient Research Partner
  • Johns Hopkins University
  • Second University of Napels
  • University of Padova
  • University Hospital Basel
  • University of Bari Aldo Moro
  • Charles University in Prague
  • University of Medicine and Pharmacy, Cluj-Napoca
  • National and Kapodistrian University of Athens
  • Policlinico San Matteo Pavia Fondazione
  • 12 de Octubre University Hospital
  • Medical University of Silesia
  • Marche Polytechnic University
  • Spedali Civili Di Brescia
  • Skåne University Hospital
  • Clinical Hospital Split
  • Azienda Ospedaliera Universitaria Integrata Verona
  • University Hospital of Infectious Diseases
  • University Hospital of Tubingen
  • Hospital General de Granollers
  • Medical University of Lublin
  • University Hospital Bonn
  • Kantonsspital Aarau
  • University Hospital of Coimbra
  • University of Niš
  • University of the Witwatersrand
  • V.A. Nasonova Research Institute of Rheumatology, RAMS
  • Maggiore Hospital Policlinico
  • University of Debrecen
  • Hospital Ramón Y Cajal
  • Norwegian Radium Hospital
  • University of Lille Nord de France
  • Lille University Hospital
  • Sapienza University of Rome
  • Carol Davila University of Medicine and Pharmacy
  • University of Münster
  • University Hospital Erlangen
  • University of Milan
  • Rheuma Clinic
  • Hospital Universitario Doctor Peset
  • Medizinsche Klinik und Poliklinik I, Dresden
  • University Hospitals Leuven
  • Saint-Luc University Hospital
  • East Tallinn Central Hospital
  • Marmara University
  • Federal University of Paraná
  • Thomas Jefferson University
  • Nicolae Testemitanu, State Medical and Pharmaceutical University
  • Centre Hospitalier de Valence
  • Dunedin School of Medicine
  • University of Cagliari
  • University of Waikato
  • St Vincent's University Hospital
  • Hospital San Juan Batista
  • Peking Union Medical College
  • Szpital Specjalistyczny im. J. Dietla
  • University Hospital Vittorio Emanuele
  • University of Bern
  • National Institute of Geriatrics, Rheumatology and Rehabilitation
  • Escola Paulista de Medicina
  • Assiut University
  • Hospital Universitario Madrid Norte Sanchinarro
  • Grenoble University Hospital
  • Rambam Medical Center
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Pages (from-to)1327-1339
JournalAnnals of the Rheumatic Diseases
Volume76
Publication statusPublished - 2017
Publication categoryResearch
Peer-reviewedYes