Update of EULAR recommendations for the treatment of systemic sclerosis

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 HunzelmannDinesh 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 Gińdzieńska-Sieskiewicz, Giovanna Cuomo, Michele Iudici, Simona Rednic, Panayiotis G. Vlachoyiannopoulos, Roberto Caporali, Patricia E. Carreira, Srdan Novak, Lorenzo Beretta, EUSTAR Coauthors

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1327-1339
Number of pages13
JournalAnnals of the Rheumatic Diseases
Issue number8
Publication statusPublished - Aug 1 2017


  • Cyclophosphamide
  • Methotrexate
  • Systemic Sclerosis
  • Treatment

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)


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