结缔组织疾病近年来认为属于全身性自身免疫性疾病范畴。这一组疾病具有类似的临床特征,常累及多个器官系统,肺部病变并非少见,少数病人肺部改变可以是该病的主要表现,往往误诊或治疗不当。本文就硬皮病、系统性红斑狼疮、类风湿性关节炎、皮肌炎、干燥综合征等病的肺部病变作一综述。硬皮病硬皮病除皮损外,并累及内脏器官,也称为进行性系统性硬化症(以下简称 PSS)。PSS 肺部有病变者约50%,Siegel 报道肺部有改变者43%,尸检则高达81%。肺部的主要病 In recent years, connective tissue diseases are considered as a systemic autoimmune disease. This group of diseases have similar clinical features, often involving multiple organ systems, lung disease is not uncommon, a small number of patients with lung changes can be the main manifestation of the disease, often misdiagnosis or improper treatment. This article reviews the pathological changes of the lungs such as scleroderma, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis and Sjogren’s syndrome. Scleroderma Scleroderma In addition to lesions, and involving the internal organs, also known as progressive systemic sclerosis (hereinafter referred to as PSS). About 50% of PSS lungs have lesions, Siegel reports 43% of lungs and 81% of autopsy cases. The main disease of the lungs