摘要目的 分析原发性气管支气管肺淀粉样变性(PTBA)的临床特点.方法 对中南大学湘雅医院2002年1月-2018年6月经病理学检查确诊的11例PTBA患者的临床症状、实验室及影像学检查、支气管镜检查及治疗等资料进行回顾性分析,并根据支气管镜下病变程度和范围对该病进行分期.结果 患者主要临床症状为咳嗽和咳痰,其次为气促和咯血;胸部CT表现为肺部渗出(4/11)、结节肿块(5/11)、间质改变(2/11),或气管支气管壁增厚(5/11)、管腔狭窄(4/11);支气管镜可见黏膜肥厚(8/11)、结节状隆起(3/11)、管腔狭窄(6/11).根据病变累及部位,1例仅累及肺部,10例累及气管和(或)支气管,伴(8/10)或不伴(2/10)肺部病变.根据镜下分期,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),弥漫性病变伴有管腔狭窄.结论 PTBA为呼吸系统罕见病,临床症状缺乏特异性,肺部影像学表现多样,支气管镜下可见气管黏膜肥厚、结节隆起,晚期患者可表现为气管狭窄.

abstractsObjective To analyze the clinical features of primary tracheobronchial pulmonary amyloidosis (PTBA).Methods 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.Results 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.Conclusions 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.