原发性支气管肺淀粉样变2例报道及文献复习

目的探讨原发性支气管淀粉样变的临床类型、病理特点、影响学特点、鉴别诊断、诊治方法及预后。方法对河南省胸科医院经病理确诊的2例结节型及弥漫型原发性支气管淀粉样变病例进行复习及总结。结果原发性支气管淀粉样变主要临床表现为咳嗽、咳痰、咳血、胸闷，影像学表现可为单发结节影或双肺弥漫型，病理切片行刚果红染色可确诊，治疗以免疫抑制剂为主。结论原发性支气淀粉样变临床表现无特异性，影像学表现多样化，误诊率高，诊断仍旧以肺穿刺或纤支镜活检取得组织行病理切片刚果红染色为确诊的最佳方法，强的松治疗效果好，预后大多数较好。

Two cases of primary bronchial amyloidosis report and review of literatures

Objective To investigate the clinical characteristics, pathological features, radiological signs, differential diagnosis, diagnosis and treatment,prognosis of primary bronchial amyloidosis（PRA）. Methods 2 case nodular and diffuse type PRA of pathologically confirmed were reviewed and summarized. Results The main clinical manifestations of PRA is cough,sputum,hemoptysis, chest tightness, There are solitary nodules or diffuse type in lung CT, Biopsy plsitive for Congo red staining can be confirmed,It,s tretment is immunosuppressive agents. Conclusions Clinical manifestations of PRA is not specificity, Imaging is diversification, Misdiagnosis rete is high, The best method of diagnosis is still to obtain lung biopsy or branchcopy biopsy tissuses for Congo red staining, Given prednisone is effective, Most patients have good prognosis.