Sharp等(1972)报告25例混合性结缔组织病,它具有系统性红斑狼疮(SLE)、硬皮病和多发性肌炎等临床特征,实验室检查抗ENA抗体阳性,有肺脏受累。Harman等报告的28例混合性结缔组织病中23例(82%)有肺功能不全。用激素治疗的14例中12例肺功能及胸片恢复正常。本文报告5例混合性结缔组织病伴有严重进行性肺损伤病人,其中3例有组织学资料并将检查结果与SLE或硬皮病肺部改变进行比较,文中还报告了皮质激素及细胞毒药物对本组病例的疗效。混合性结缔组织病临床特征为多关节痛、两手弥漫性肿胀、雷诺氏现象、食道运动低下和炎症性肌 Sharp et al. (1972) reported 25 cases of mixed connective tissue disease with clinical features such as systemic lupus erythematosus (SLE), scleroderma and polymyositis. Laboratory tests were positive for anti-ENA antibody with lung involvement. Of the 28 patients with mixed connective tissue disease reported by Harman et al, 23 (82%) had pulmonary insufficiency. Of 14 cases treated with hormone, 12 cases of lung function and chest X-ray returned to normal. This article reports 5 patients with mixed connective tissue disease with severe progressive lung injury, 3 patients with histological data and the examination results and lung changes in SLE or scleroderma comparison, also reported in the corticosteroid and cytotoxic The efficacy of drugs in this group of patients. Clinical features of mixed connective tissue disease are polyarticular pain, diffuse swelling of both hands, Raynaud’s phenomenon, low esophageal motility, and inflammatory muscle