Mediterr J Rheumatol 2015; 26(1): 56-63
Evidence-based treatment for systemic sclerosis
The treatment of systemic sclerosis is fraught with many challenges. The therapeutic approach targets affected organs, since there is no medications that modify the disease itself. In this review we tried to present the therapeutic management of main manifestations of systemic sclerosis based on the EULAR-EUSTAR recommendations and published data.  In particular, in skin involvement, serositis, or arthritis, methotrexate along with small dose of corticosteroids can be used.  For Raynaud’s phenomenon and digital ulcers, high doses of calcium channel blockers, and inrefractory cases, IV prostacyclin analogues are used. For the prevention of digital ulcers, bosentan can be used. Pulmonary arterial hypertension is managed according to functional stage with inhibitors of endothelin receptors, inhibitors of phosphodiesterase-5, prostacyclin analogues, or their combination.  For interstitial lung disease, cyclophosphamide IV or per os with or without corticosteroids are used for 6-12 months.  As maintenance treatment azathioprine or mycophenolate is used. For sceroderma renal crisis, inhibitors of angiotensin converting enzyme are used. For gastrointestinal manifestations, proton pump inhibitors, domperidone, cyclic administration of antibiotics or octreotide are used. The therapeutic choice for a particular patient is based on clinical judgement.