系统性硬化症肺间质疾病的治疗.pdfVIP

系统性硬化症肺间质疾病的治疗* 于一云 1,2** 付思思 1,2 薛愉 1, 2 邹和建 1,2*** 译 （1. 复旦大学附属华山医院风湿科 上海 200040 ；2. 复旦大学风湿、免疫、过敏性疾病研究中心 上海 200040） 摘 要 系统性硬化症（SSc）在结缔组织病中死亡率最高，以血管损害、皮肤和各种内脏器官的炎症和纤维化为特点。 间质性肺病（ILD）常常使 SSc 的临床表现复杂化使疾病恶化，预后差。ILD 是 SSc 中最主要的死因，SSc-ILD 患者的 管理具有极大挑战。根据肺功能下降及高分辨率 CT 早期诊断间质性肺炎是管理中最重要的一环。这篇文章总结了 SSc- ILD 分类、病理、诊断、预后、生存、目前及今后的一些治疗方法。 关键词 系统性硬化症 硬皮病 间质性肺病 纤维化 治疗 R593.25 A 1006-1533(2017)S1-0037-08 中图分类号 ： 文献标识码 ： 文章编号 ： Treatment of pulmonary interstitial disease in systemic sclerosis* YU Yiyun1,2**, FU Sisi1,2, XUE Yu1,2, ZOU Hejian1,2***, Translate (1. Department of Rheumatism, Huashan Hospital, Fudan University, Shanghai 200040, China; 2. Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China) ABSTRACT Systemic sclerosis (SSc) has the highest mortality in connective tissue disease characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) often complicates the clinical manifestations of SSc, leading to disease progression and poor prognosis. ILD is the most important cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early diagnosis of interstitial pneumonia according to decreased lung function and high resolution CT is one of the most important aspects of management. This article summarizes the SSc-ILD classification, pathology, diagnosis, prognosis, survival, current and future treatment options. KEY WORDS systemic sclerosis; scleroderma; interstitial lung disease; fibrosis; treatment pulmonary arterial hypertension