长期误诊的原发性气管支气管淀粉样变

目的：探讨原发性气管支气管淀粉样变的临床症状和诊断方法。方法：分析1例被误诊多年的原发性气管支气管淀粉样变病例的临床表现和诊断经过。结果：本例患者为68岁男性，30多年前开始出现反复咳嗽、咳痰以及渐进性的呼吸困难，曾多次在外院就诊，诊断为慢性支气管炎、支气管扩张以及支气管结核，反复给与抗生素治疗，并曾行抗结核治疗3个月，但治疗效果差，患者症状逐渐加重。患者来中南大学湘雅医院就诊后，经完善支气管镜检查后病理确诊为原发性气管支气管淀粉样变。结论：气管支气管淀粉样变是一种气管和支气管黏膜下异常的淀粉样物质沉积所导致的罕见病，临床症状多样，常被误诊为其他肺部疾病。支气管镜检查是诊断气管支气管淀粉样变的重要方法，病理学检查是诊断的金标准，对于不明原因慢性咳嗽患者，建议及早行支气管镜检查。

Delayed diagnosis for primary tracheobronchial amyloidosis

Objective: To determine clinical features and diagnostic methods for primary tracheobronchialamyloidosis (TBA).Methods: Th e clinical manifestations and diagnosis of a male patient who had been misdiagnosedfor many years were described and analyzed.Results: Th e patient was a 68-year-old male who complained of recurrent cough, expectoration,and progressive dyspnea for more than 30 years. He had been diagnosed with chronic bronchitis,bronchiectasis, and endobronchial tuberculosis in other hospitals and treated with antibioticsfrequently and anti-tubercular agents for 3 months. Despite the treatments, the patient’s symptomswere progressively worse. Finally, he came to Xiangya Hospital, Central South University, and wasclearly diagnosed with primary TBA based on histopathological evidence aft er bronchoscopy.Conclusion: TBA, a rare disease resulting from abnormal submucosal amyloid deposition in the tracheaand bronchi, may display with many diff erent symptoms. TBA is oft en misdiagnosed with other pulmonarydiseases. Th e use of bronchoscopic techniques is essential for the diagnosis of TBA. Histopathology remainsthe gold standard for diagnosis of primary TBA. So, for patients with chronic cough of unknown etiology,bronchoscopy should be performed to obtain biopsy samples for the defi nitive diagnosis.