原发性气管、支气管淀粉样变合并肺隐球菌病1例并文献复习

Primary tracheal trocheohronehiay amyioidosis with case report and literature review

目的分析气管、支气管淀粉样变（TBA）的临床特征、实验室检查以及影像学和电子气管镜检查结果，明确其诊断，提高临床呼吸科医师对该病的认识。方法报道1例明确诊断的TBA患者，其临床表现、辅助检查及诊治经过，并结合文献进行分析。结果患者，男性，68岁，因咳嗽、咳大量白黏痰伴气喘人院，其生化、血常规检查均正常，高分辨胸部CT示：气管管壁增厚，电子气管镜检查：均见气管、支气管管腔狭窄，黏膜增生充血水肿，见无蒂小结节，黏膜活检，刚果红染色阳性。参照波士顿大学医学院淀粉样变治疗和研究项目组指定的原发性TBA诊断标准，确诊为TBA。治疗过程中因较大剂量使用肾上腺皮质激素和抗生素，并发了隐球菌感染。结论原发性气管、支气管淀粉样变极为罕见，临床表现和影像学特征缺乏特异性，极易误诊和漏诊，最终依靠电子支气管镜，病理活检，刚果红染色阳性，排除全身淀粉样变的证据，才能确诊，密切注意治疗过程中的并发症。

Objective To analysis the clinical features, laboratory tests, imaging findinds and endoscopic manifestations of trocheohronehiay amyyioidosis （TBA） to clear the diagnosis,improve clinical physician% understangding of the disease. Methods One case of a patient diagnosed TBA was reported, its clinical informtion,laboratory examinations, course of the diagnosis and treatment and analyzing with relative literatures were observed. Results The patient, male, 68 years old, in our hospital was admitted due to ＂cough, spit a lot of white thick sputum, short breath＂, all laboratory tests were normal, highresolution spiral CT chest CT showed the tracheobronchial wall was thick,brochoscopy found the airway wall was thick and narrow, mucosal congestion and edema, sessile nodules, then mucosal biopsy, congo red stain （＋）. Based on the Boston University Medicine Academy amyloidosis treatment and research project team formulated the primary trocheohronehiay amyyioidosis diagnostic criteria, to be diagnosed TBA. Becaused of using high-dose adrenocorticotropic hormone through the treament, it led to cryptococcal infection. Conclusions Tracheobronchial amyloidosis is rare in clinic and is proven to be easily misdiagnosed,because clinical manifestations and imaging features were lack of specificity. It can be diagnosied eventually rely on electronic bronchoscope, pathological biopsy, congo red stain （ ＋ ）, rule out the evidence to diagnosis of systemic amyloidosis, besides, close attention should be payed to the complication in the process of the treatment.