Systemic sclerosis (SSc) is a chronic, multisystemic, connective tissue disease characterized by fibrosis of the skin and visceral organs and the impairment of blood vessels. The etiology of SSc is unknown and its pathogenesis unclear. Effective treatment of SSc is also unavailable. Cyclophosphamide (CFX) has been reported to exert some beneficial actions in SSc patients, in particular in those with interstitial lung disease. However, it should be kept in mind that CFX treatment is related to multiple side effects. The aim of this study was to review available literature concerning the use of CFX in SSc patients.
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