Lü X, Luo T, Liu L, Zhou H, Meng J
Department of Respiratory and Critical Care Medicine, National Clinical Research Center for Respiratory Disease, Xiangya Hospital, Central South University, Changsha 410000, China.
Department of Respiratory and Critical Care Medicine, National Clinical Research Center for Respiratory Disease, Xiangya Hospital, Central South University, Changsha 410000, China （is working in the Department of Respiratory Medicine, Central Hospital of Shaoyang, Shaoyang 422000, China）.
Zhonghua Yi Xue Za Zhi. 2019 Mar 26;99(12):918-922. doi: 10.3760/cma.j.issn.0376-2491.2019.12.008.
To analyze the clinical features of primary tracheobronchial pulmonary amyloidosis (PTBA). The records of 11 patients with PTBA diagnosed by pathology from January 2002 to June 2018 in Xiangya Hospital of Central South University were retrospectively reviewed, including clinical manifestations, laboratory and imaging examination, bronchoscopic manifestations and therapies. Meanwhile, PTBA was staged based on the severity and extent of lesion under bronchoscopy. The most common clinical symptoms were cough and expectoration followed by shortness of breath and hemoptysis. Chest computed tomography (CT) revealed pulmonary infection (4/11), pulmonary nodules and masses (5/11), interstitial lesions (2/11), tracheal bronchial wall thickening (5/11) or airway stenosis (4/11). Bronchoscopy showed mucosal hypertrophy (8/11), nodular bulge (3/11), and luminal stenosis (6/11). According to the lesion involvement, 1 case only involved the lungs, 10 cases involved the trachea and/or bronchus, with (8/10) or without (2/10) lung lesions. According to the bronchoscopic staging, 2 cases (2/10) in the stage Ⅰ, lesions were limited; 2 cases (2/10) in the stage Ⅱ, lesions were diffuse; 6 cases (6/10) in the stage Ⅲ, diffuse lesions with stenosis. PTBA is a rare disease of the respiratory system, with unspecific clinical manifestations and diverse pulmonary imaging findings; airway mucosal hypertrophy and nodular bulging can be seen under bronchoscopy. Patients with advanced stage may present with airway stenosis.
分析原发性气管支气管肺淀粉样变性（PTBA）的临床特征。回顾性分析2002年1月至2018年6月在中南大学湘雅医院经病理确诊的11例PTBA患者的病历，包括临床表现、实验室及影像学检查、支气管镜表现及治疗情况。同时，根据支气管镜下病变的严重程度和范围对PTBA进行分期。最常见的临床症状为咳嗽、咳痰，其次为气短和咯血。胸部计算机断层扫描（CT）显示肺部感染（4/11）、肺结节及肿块（5/11）、间质性病变（2/11）、气管支气管壁增厚（5/11）或气道狭窄（4/11）。支气管镜检查显示黏膜肥厚（8/11）、结节样隆起（3/11）及管腔狭窄（6/11）。根据病变累及情况，1例仅累及肺部，10例累及气管和/或支气管，其中8例（8/10）合并肺部病变，2例（2/10）不合并肺部病变。根据支气管镜分期，Ⅰ期2例（2/10），病变局限；Ⅱ期2例（2/10），病变弥漫；Ⅲ期6例（6/10），弥漫性病变伴狭窄。PTBA是一种罕见的呼吸系统疾病，临床表现无特异性，肺部影像学表现多样；支气管镜下可见气道黏膜肥厚及结节样隆起。晚期患者可出现气道狭窄。
