干燥综合征的肺部病变  

Pulmonary involvement in Sj?gren's syndrome

作　　者：李霞[1,2] 费允云[1] 张烜[1] 

机构地区：[1]北京协和医院风湿免疫科 [2]首都医科大学宣武医院风湿免疫科

出　　处：《中国实用内科杂志》2017年第6期484-487,共4页Chinese Journal of Practical Internal Medicine

摘　　要：干燥综合征(Sj?gren’s syndrome,SS)腺体外系统受累较常见,包括肺部病变。SS肺损害定义为出现呼吸道症状、肺功能异常或影像学异常等。患者表现各异,支气管炎和支气管扩张是常见的气道病变。SS合并间质性肺疾病是常见的肺部病变。另外,SS患者有患肺淋巴瘤的高风险,也可出现肺动脉高压、肺囊性病变、肺淀粉样变及胸膜受累等。基线评估SS患者肺部病变是必要的。目前关于SS合并肺损害的治疗尚缺乏大型、随机对照临床研究数据。进展性肺损害患者,糖皮质激素和(或)免疫抑制剂治疗是必要的。SS-肺动脉高压的治疗目前推荐靶向药物治疗为主。Extraglandular organ systems of Sj6gren＇s syndrome （SS）may frequently be involved, including the respiratory system. Pulmonary involvement is defined by respiratory symptoms, pulmonary function or radiographic abnormalities. Clinical manifestations vary. Bronchiolitis and bronchiectasis are the most common airway manifestations. Interstitial lung disease is the most common lung disease of SS. Patients with SS are at high risk of lung lymphoma. Other lung abnormalities include pulmonary artery hypertension （PAH）, pulmonary cystic lesions, amyloidosis, and pleural disease. Corticosteroids alone or in combination with other immunosuppressors are quite necessary for the patients with progressive chest symptoms. PAH targeted therapy is recommended in SS-PAH patients.

关 键 词：干燥综合征 气道病变 间质性肺病 肺动脉高压 

分 类 号：R593.2[医药卫生—内科学]