Systemic sclerosis Chronic management Management of digital ulcerations EULAR Q Does the patient have digital ulcerations? Yes Consider phosphodiesterase type 5 inhibitors to treat digital ulcerations in patients with SSc. B Consider intravenos iloprost to treat digital ulcerations in patients with SSc. B No Management of digital ulcerations EULAR Q Does the patient have multiple digital ulcerations despite the use of CCBs, PDE-5 inhibitors, or iloprost? Yes Consider bosentan to reduce of the number of new digital ulcerations in patients with SSc, especially in patients with multiple digital ulcerations despite use of CCBs, phosphodiesterase type 5 inhibitors or iloprost. B No Management of gastrointestinal disease Q Does the patient have systemic sclerosis related gastro-oesophageal reflux? Yes Administer PPIs for SSc-related gastro-esophageal reflux disease and for the prevention of esophageal ulcers and strictures. B No Management of gastrointestinal disease Q Does the patient have systemic sclerosis related symptomatic GI motility disturbances? Yes Offer prokinetic drugs for the management of SSc-related symptomatic motility disturbances, such as dysphagia, dyspepsia, early satiety, bloating, and pseudo- obstruction. B No Management of gastrointestinal disease Q Does the patient have symptomatic small intestine bacterial overgrowth? Yes Consider intermittent or rotating antibiotics in patients with symptomatic SIBO. C No Management of interstitial lung disease Q Does the patient have ILD? Yes Consider cyclophosphamide in patients with SSc-related ILD, in particular for patients with progressive disease. B No Management of pulmonary hypertension Q Does the patient have systemic sclerosis related pulmonary arterial hypertension? Yes Consider endothelin receptor antagonists, phosphodiesterase type 5 inhibitors or riociguat to treat SSc-associated pulmonary arterial hypertension. C No Management of pulmonary hypertension Q Does the patient have severe systemic sclerosis related pulmonary arterial hypertension (class III and IV)? Yes Consider IV epoprostenol in patients with severe SSc-related pulmonary arterial hypertension. B No Management of Raynaud's phenomenon Q Does the patient have systemic sclerosis associated Raynaud's phenomenon? Yes Consider dihydropiridine-type calcium antagonists, usually oral nifedipine, as first-line therapy for SSc-associated Raynaud's phenomenon. B No Management of Raynaud's phenomenon Q Does the patient have severe systemic sclerosis-associated Raynaud's phenomenon? Yes Consider intravenous iloprost for severe SSc-associated Raynaud's phenomenon. B No Management of skin disease Q Does the patient have skin manifestations of early diffuse systemic sclerosis? Yes Consider methotrexate for the treatment of skin manifestations in early diffuse SSc. B No Hematopoietic stem cell transplantation Q Does the patient have rapidly progressive systemic sclerosis at risk of organ failure? Yes Consider HSCT for the treatment of selected patients with rapidly progressive SSc who are at risk of organ failure. B