结缔组织病相关性间质性肺疾病的诊治进展

结缔组织病（CTD）是一组累及全身多系统的自身免疫病，合并间质性肺疾病（ILD）较为常见，导致肺功能障碍、低氧血症，严重影响患者的生活质量和预后。不同CTD显示出异质性的ILD类型。CTD-ILD常见的影像学和组织病理学类型是非特异性间质性肺炎、机化性肺炎和普通型间质性肺炎。大部分CTD-ILD表现为缓慢进展的肺损伤，少数患者短期内急剧进展且预后不良。CTD-ILD目前主要应用糖皮质激素和／或免疫抑制剂治疗，生物制剂显示出临床疗效，尚需随机临床试验证实。部分ILD患者具有自身免疫病的特征，不足以诊断为某种特定的CTD，应予以密切随访。

Update of diagnosis and treatment in connective tissue disease associated interstitial lung disease

Connective tissue disease （CTD） is commonly associated with interstitial lung disease （ILD） resulting in respiratory dysfunction and even hypoxemia, and severely affecting the patients＇ quality of life and prognosis. The various CTD show the heterogeneity of the different ILD patterns. The most common patterns of ILD on radiology and pathology are nonspecific interstitial pneumonia, organizing pneumonia,and usual interstitial pneumonia. The majority of CTD-ILD manifests chronic lung injury, while the condition of minor patients processes rapidly in a short period leading to poor prognosis. Immunosuppressive therapy remains the mainstream for CTD-ILD meanwhile the randomized clinical trials are developing to determine optimal treatment strategies for the distinct forms of CTD-ILD. ILD patients with the features of autoimmune diseases should follow up and select proper treatment options.