Is there still a role for cyclophosphamide in the treatment of systemic sclerosis?

Cyclophosphamide has been the cornerstone of treatment of systemic sclerosis for a long time and is the first-choice therapy for treating systemic sclerosis–associated interstitial lung disease according to the European League Against Rheumatism recommendations on treatment of systemic sclerosis. However, new therapeutic options are emerging, and treatment with cyclophosphamide is hampered by its toxicity and restricted possible treatment duration. This review has a focus on the evidence of efficacy of cyclophosphamide in different aspects of systemic sclerosis and its organ involvements, reviews its toxicity, and will answer the question whether there is still a role for cyclophosphamide in the treatment of systemic sclerosis, taking the evidence and current therapeutic options into account.