系统性硬化症肺部病变的临床特征及病理特点

目的 :了解系统性硬化症 (SSc)患者肺部病变的临床特征及病理特点。方法 :对确诊的 6 8例 SSc进行回顾性临床分析。结果 :5 7例 (83.8% )有程度不同的胸部 X线异常 ,4 4例 (6 4 .7% )有明显肺纤维化征象 ,5例无肺纤维化征象而以肺动脉高压和 (或 )心衰为突出表现。14例死亡主要原因为呼吸衰竭及心功能不全。6 8例中有 6例伴发恶性肿瘤。结论 :SSc肺损害发生率高 ,肺纤维化和肺功能不全进程与患者预后密切相关。

Clinicopathological Studies of Pulmonary Involvement of Patients with Systemic Sclerosis

Objective:To assess the incidence of pulmonary lesions of patients with systemic sclerosis(SSc) as well as its clinical and pathological changes.Methods:The pulmonary lesions was studied clinicopathologically in 68 patients who were compatible with the diagnosis of SSc.Results:There were 57 (83.8%) cases with the abnormal change of different extension in the roentgen of chest, 44 (64.7%) with significant pulmonary fibrosis,six with the malignant tumor, five without pulmonary fibrosis but with marked pulmonary hypertension and/or cardiac failure,and 14 died of the distinct cardiopulmonary involvement among them.Conclusion:Morbidity of pulmonary lesions of patients with SSc is significantly increased.The course of pulmonary fibrosis and pulmonary insufficiency of patients with SSc is closely related with their prognosis.