State-of-the-art evidence in the treatment of systemic sclerosis

Systemic sclerosis (SSc) is a rare autoimmune disease that affects multiple organs and involves fibrosis and vasculopathy. Treatment options for SSc have improved, including early diffuse cutaneous SSc (dcSSc) with the use of immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab, and tocilizumab. Autologous haematopoietic stem cell transplantation may be beneficial for patients with rapidly progressive early dcSSc. Morbidity from interstitial lung disease and pulmonary arterial hypertension is improving with the use of proven therapies such as mycophenolate mofetil and combination therapy for pulmonary arterial hypertension. Raynaud phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers, phosphodiesterase 5 inhibitors, and intravenous iloprost. Research is needed to develop more effective treatments, best practices for organ-specific screening and early intervention, and sensitive outcome measurements.