亟待解决的问题：系统性硬化症中是否使用环磷酰胺。
The burning question: To use or not to use cyclophosphamide in systemic sclerosis.
作者信息
Bruni Cosimo, Furst Daniel E
机构信息
Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.
Division of Rheumatology, Department of Medicine, University of California Los Angeles, Los Angeles, CA, USA.
出版信息
Eur J Rheumatol. 2020 Oct;7(Suppl 3):S237-S241. doi: 10.5152/eurjrheum.2020.19104. Epub 2020 Oct 1.
Abstract
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.
摘要
纤维化、炎症和血管病变是系统性硬化症（SSc）发病机制的主要决定因素。环磷酰胺（CYC）作为一种烷化剂，多年来一直用于治疗SSc中的皮肤纤维化和间质性肺病，并且仍是造血干细胞移植的主要药物。尽管CYC在减轻肺功能损害和皮肤紧绷方面有显著效果，但它存在重大的安全负担，包括感染风险以及骨髓和膀胱毒性。此外，它会影响生育能力，还会增加未来患癌风险，尤其是膀胱癌。本综述总结了目前关于使用CYC治疗SSc的证据、其疗效和安全性概况，以及目前可用于或正在试验的针对SSc肺部和皮肤受累的替代药物。
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