系统性硬化症相关间质性肺病的治疗

Treatment of Systemic Sclerosis-related Interstitial Lung Disease

系统性硬化症是一种可累及全身多个系统的结缔组织病,预后较差,死亡率较高。肺部受累引起间质性肺病是系统性硬化症患者常见的死亡原因。国内外尚无系统性硬化症相关间质性肺病的诊疗指南,环磷酰胺是目前唯一治疗该病的一线药物,然而,环磷酰胺的毒副作用限制了其应用。近年来,该病的治疗受到国内外学者越来越多的关注,本文就系统性硬化症相关间质性肺病的治疗策略做一总结,并对新兴的治疗方案进行重点阐述。

Systemic sclerosis is a connective tissue disease that affects multiple systems. It has a poor prognosis and a high mortality rate. Interstitial lung disease is a common cause of death in patients with systemic sclerosis. There is no guidance for the diagnosis and treatment of systemic sclerosis-associated interstitial lung disease. Cyclophosphamide is currently the only first-line treatment for this disease. However, the benefits of the cyclophosphamide are affected by its complex adverse events. In recent years, more and more researchers pay attention to the treatment of this disease,we made this review to summarize the treatment strategies for systemic sclerosis-associated interstitial lung disease, and focus on emerging treatment options.