European Journal of Rheumatology
Invited Review

The burning question: To use or not to use cyclophosphamide in systemic sclerosis


Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy


Department of Medicine, University of Washington, Seattle, Washington, USA

Eur J Rheumatol 2020; 7: Supplement S237-S241
DOI: 10.5152/eurjrheum.2020.19104
Read: 2186 Downloads: 1032 Published: 27 October 2020

Fibrosis, inflammation, and vasculopathy are the main determinants of systemic sclerosis (SSc) pathogenesis. Cyclophosphamide (CYC), an alkylating agent, has been used to treat skin fibrosis and interstitial lung diseases in SSc for many years and still represents a mainstay in hematopoietic stem cell transplantation. Despite significant effect in reducing lung functional impairment and skin tightness, CYC has a significant safety burden, including infection risk and bone marrow and bladder toxicity. Moreover, it can affect fertility and also cause a predisposition for cancer development in the future, particularly in the bladder. This review summarizes the current evidences regarding the use of CYC to treat SSc, its efficacy and safety profile, and currently available or tested alternative drugs for lung and skin involvement in SSc.

Cite this article as: Bruni C, Furst DE.  The burning question: To use or not to use cyclophosphamide in systemic sclerosis. Eur J Rheumatol 2020; 7(Suppl 3): S237-41.

EISSN 2148-4279