原发性支气管肺淀粉样变是一种不可溶性淀粉样蛋白物质在气管、支气管及肺组织中沉积而表现各异的临床综合征,属少见病,且临床表现缺乏特异性,诊断主要依靠组织活检病理学检查。病程后期可出现支气管狭窄,引发呼吸困难及咯血等症状,预后差,目前尚无特异性治疗,故早诊断、早治疗对本病预后有所帮助。 Primary bronchial pulmonary amyloidosis is a clinical syndrome characterized by the deposition of insoluble amyloid in the trachea, bronchus and lung tissue. It is a rare disease characterized by a lack of specificity in clinical manifestations. Diagnosis mainly depends on the organization Biopsy pathology. The latter part of the course of the disease may have bronchial stenosis, causing dyspnea and hemoptysis and other symptoms, the prognosis is poor, there is no specific treatment, so early diagnosis and early treatment of the prognosis of this disease is helpful.