气管支气管淀粉样变9例临床分析

Retrospective analysis of nine cases of tracheobronchial amyloidosis

目的分析气管支气管淀粉样变(TBA)的临床特点及诊疗情况,提高对该病的认识及诊疗水平。方法回顾性分析2011年1月至2017年4月郑州大学第一附属医院收治的9例气管支气管淀粉样变性患者的临床资料。结果临床表现:胸闷3例,咳嗽4例,咯血2例,发热1例,声音嘶哑1例。胸部CT检查:2例患者结果报告肺门占位并肺叶不张及实变,4例结果报告气管及支气管狭窄,1例结果报告气道内不规则软组织影,2例结果报告气管支气管未见异常。诊断:5例经首次纤维支气管镜活检即确诊,1例手术确诊,3例多次气管镜活检后确诊,气管镜下可见管壁呈弥漫增厚型、气道新生物型、局部水肿狭窄阻塞型。治疗:1例T-SPOT弱阳性及1例结明三项弱阳性者接受抗结核药联合强的松治疗,1例症状加重,1例失访;4例给予激素治疗,2例观察中,1例自行停药,1 a后复查气道弥漫重度狭窄,伴发喉鸣音,另1例规律复查;1例手术切除;2例对症治疗。结论气管支气管淀粉样变的临床表现主要为呼吸系统症状,缺乏特异性,易误诊,纤维支气管镜检查是诊断该病的主要手段,胸部CT能起到一定的筛查作用,临床主要根据具体病变部位及程度选择合适的治疗方法。

Objective To analyze the clinical features and diagnosis and treatment of tracheobronchial amyloidosis and improve the understanding and diagnosis and treatment of the disease.Methods The clinical data of nine patients with tracheobronchial amyloidosis who were admitted to the First Affiliated Hospital of Zhengzhou University from January of 2011 to April of 2017 were retrospectively analyzed.Results Clinical manifestations:three cases of chest tightness,four cases of cough,two cases of hemoptysis,one case of fever,one case of hoarseness.Chest CT examination:hilar occupancy and lobular atelectasis and consolidation in two patients,tracheal and bronchial stenosis in four patients,irregular soft tissue within the airway in one patient,and no abnormality in the tracheobronchial tube in two patients.Diagnosis:five cases were diagnosed by fiberoptic bronchoscopy for the first time,one case was diagnosed by operation,three cases were diagnosed by multiple bronchoscope biopsy,including bronchial wall diffuse thickened type,new airway type,narrow type of local edema and obstruction.Treatment:one case of weak positive T-SPOT responses and one case of weak positive Mycobacterium tuberculosis specific cellular immune response were given anti-TB drugs combined with prednisone treatment,then the symptoms were aggravated in one patient and one case was lost after treatment;four cases were given hormone therapy,then two patients were in observation,one patient discontinued treatment on his own and the examination showed diffuse severe stenosis of the airway accompanied by laryngeal sounds after one year,and another one was reviewed regularly;one patient underwent surgical resection;two patients were symptomatically treated.Conclusions The clinical manifestations of tracheobronchial amyloidosis mainly are respiratory symptoms,lack of specificity,easy to misdiagnosis.Bronchoscopy is the main mean of diagnosis of the disease,and chest CT can play a role in screening.The suitable treatment method is chosen according to the specific location and degree of the disease.