Journal Article

Update of EULAR recommendations for the treatment of systemic sclerosis

Frerix, Marc, Smith, Vanessa, Wigley, Frederic, van den Hoogen, Frank, Hunzelmann, Nicolas, Khanna, Dinesh, del Galdo, Francesco, Cutolo, Maurizio, Kulak, Agnieszka, Avouac, Jerome, Denton, Christopher P, Furst, Daniel E, Czirjak, Laszlo, Zulian, Francesco, Valentini, Gabriele, Becker, Mike, Damjanov, Nemanja, Figelstone, Kim, Allanore, Yannick, Distler, Oliver, Kowal-Bielecka, Otylia, Müller-Ladner, Ulf, Sulli, Alberto, Tarner, Ingo, van Laar, Jacob M, Matucci-Cerinic, Marco, Riemekasten, Gabriela, Guiducci, Serena, Walker, Ulrich A, Distler, Jörg H W, Welling, Joep, Clements, Philip, Silver, Richard, Tyndall, Alan, Fransen, Jaap, Foeldvari, Ivan, Herrick, Ariane L

2017

Overview

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.

• Publisher: Elsevier Limited
• Subject: Angiotensin-Converting Enzyme Inhibitors - therapeutic use / Clinical Medicine / Clinical trials / Delphi Technique / Endothelin Receptor Antagonists - therapeutic use / Endothelins / Europe / Experts / Fingers / Fluoxetine / Fluoxetine - therapeutic use / Gastrointestinal Diseases - etiology / Gastrointestinal Diseases - therapy / Glucocorticoids - therapeutic use / Hematopoietic Stem Cell Transplantation / Hematopoietic stem cells / Humans / Hypertension, Pulmonary - etiology / Hypertension, Pulmonary - therapy / Kidney Diseases - etiology / Kidney Diseases - therapy / Klinisk medicin / Literature reviews / Lung diseases / Lung Diseases - etiology / Lung Diseases - therapy / Medical and Health Sciences / Medicin och hälsovetenskap / Patients / Phosphodiesterase / Phosphodiesterase 5 Inhibitors - therapeutic use / Prostacyclin / Prostaglandins I - therapeutic use / Pulmonary hypertension / Pyrazoles - therapeutic use / Pyrimidines - therapeutic use / Raynaud Disease - etiology / Raynaud Disease - therapy / Rheumatology / Scleroderma / Scleroderma, Systemic - complications / Scleroderma, Systemic - therapy / Selective Serotonin Reuptake Inhibitors - therapeutic use / Skin diseases / Stem cell transplantation / Studies / Systemic sclerosis / Transplantation / Ulcer - etiology / Ulcer - therapy / Ulcers