原发性支气管肺淀粉样变1例并文献复习  

Primary bronchial and pulmonary amyloidosis：a report of a case and the literature review

作　　者：穆善善 魏海东[2] Mu Shanshan;Wei Haidong(Department of Gastroenterology,Second Hospital of Lanzhou University,Lanzhou 730030,China)

机构地区：[1]兰州大学第二医院消化内科,730030 [2]兰州大学第二医院呼吸科,730030

出　　处：《国际呼吸杂志》2018年第17期1326-1330,共5页International Journal of Respiration

摘　　要：目的讨论原发性支气管肺淀粉样变的病因、发病机制、临床表现、诊断、治疗及预后。方法对我院确诊的1例和2000-2017年国内文献报道的56例原发性支气管肺淀粉样变进行回顾性分析。结果①症状多为咳嗽、咳痰、气促、咯血；②胸片以肺纹理增粗为主，CT及支气管镜检查均以支气管壁增厚、管腔狭窄常见；③肺功能以阻塞性通气功能障碍为主；④病变组织经刚果红染色呈阳性；⑤治疗主要有介入、化疗、外科手术及对症治疗等。结论该病临床表现无特异性，确诊需病理检查及刚果红染色，目前尚无公认治疗方案，早诊断、早治疗对预后有一定作用。Objective To investigate the etigology, pathogenesis, clinical features, diagnosis, treatment and prognosis of primary bronchial and pulmonary amyloidosis. Methods We retrospectively studied 56 primary bronchial and pulmonary amyloidosis cases reported in Chinese literature during 2000- 2017 and one in our hospital. Results （1）The main clinical manifestations included cough, sputum, short breath and hemoptysis. （2）Thickening of lung markings was the most common chest X-ray;Airway wall thickening and stenosis was the most common CT manifestation and endoscopic finding.（3）Pulmonary function test was mainly obstructive ventilation function. （4）A mean eosinphilic deposit in bronchus and lung after Haematokylin and Eosin （HE） staining of the lesion, the Congo red staining positive. （5） The treatments of the disease included intervention, chemotherapy, surgery and so on. Conclusions Primary bronchial and pulmonary amyloidosis is rare, correct diagnose needs pathological examination and there is no specific treatment for it currently.

关 键 词：淀粉样变 支气管 诊断 治疗 预后 

分 类 号：R562.2[医药卫生—呼吸系统]