2016 | 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 Fegelstone, 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 Sulli, Alan Tyndall, Joep Welling, Frederic Wigley, Gabriele Valentini, Ulrich A Walker, EUSTAR Coauthors
The European League against Rheumatism (EULAR) has updated its 2009 recommendations for the treatment of systemic sclerosis (SSc). The update was based on new evidence and expert consensus, involving 32 SSc specialists, two patients, and a clinical epidemiologist. The task force selected 46 clinical questions related to 26 interventions for systematic review. The new recommendations include 16 guidelines addressing various SSc-related organ complications, such as Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis (SRC), and gastrointestinal involvement.
For RP, phosphodiesterase type 5 (PDE-5) inhibitors and dihydropyridine-type calcium antagonists are recommended as first-line treatments. Intravenous iloprost is recommended for severe RP. For DUs, intravenous iloprost and PDE-5 inhibitors are recommended. Bosentan is recommended for reducing new DUs, especially in patients with multiple DUs despite other treatments. For PAH, endothelin receptor antagonists (ERA), PDE-5 inhibitors, and riociguat are recommended. Intravenous epoprostenol is recommended for severe PAH.
For skin and lung disease, methotrexate is recommended for early diffuse SSc. Cyclophosphamide (CYC) is recommended for SSc-related interstitial lung disease (SSc-ILD), particularly in patients with progressive lung disease. Haematopoietic stem cell transplantation (HSCT) is recommended for selected patients with rapidly progressive SSc at risk of organ failure.
The recommendations emphasize evidence-based treatment and consider the balance between efficacy and toxicity. They also highlight the importance of individualized treatment and future research directions. The updated recommendations aim to guide rheumatologists in managing SSc patients and support future clinical research.The European League against Rheumatism (EULAR) has updated its 2009 recommendations for the treatment of systemic sclerosis (SSc). The update was based on new evidence and expert consensus, involving 32 SSc specialists, two patients, and a clinical epidemiologist. The task force selected 46 clinical questions related to 26 interventions for systematic review. The new recommendations include 16 guidelines addressing various SSc-related organ complications, such as Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis (SRC), and gastrointestinal involvement.
For RP, phosphodiesterase type 5 (PDE-5) inhibitors and dihydropyridine-type calcium antagonists are recommended as first-line treatments. Intravenous iloprost is recommended for severe RP. For DUs, intravenous iloprost and PDE-5 inhibitors are recommended. Bosentan is recommended for reducing new DUs, especially in patients with multiple DUs despite other treatments. For PAH, endothelin receptor antagonists (ERA), PDE-5 inhibitors, and riociguat are recommended. Intravenous epoprostenol is recommended for severe PAH.
For skin and lung disease, methotrexate is recommended for early diffuse SSc. Cyclophosphamide (CYC) is recommended for SSc-related interstitial lung disease (SSc-ILD), particularly in patients with progressive lung disease. Haematopoietic stem cell transplantation (HSCT) is recommended for selected patients with rapidly progressive SSc at risk of organ failure.
The recommendations emphasize evidence-based treatment and consider the balance between efficacy and toxicity. They also highlight the importance of individualized treatment and future research directions. The updated recommendations aim to guide rheumatologists in managing SSc patients and support future clinical research.