原发性支气管肺淀粉样变性10例临床分析

Primary bronchial and pulmonary amyloidosis:a clinical analysis of 10 cases

目的：探讨原发性支气管肺淀粉样变性临床表现、诊断、治疗及预后。方法回顾性分析10例原发性支气管肺淀粉样变性患者的临床特点。结果原发性支气管肺淀粉样变性患者临床表现为咳嗽、咳痰、咯血、气促，可经纤维支气管镜、肺穿刺或胸腔镜手术活检病理检查确诊，刚果红染色阳性为典型病理特征。结论本病临床少见，病理检查为确诊金标准。目前无疗效确切的治疗药物，气管受累及弥漫性病变者预后差。

Objective To study the manifestation,diagnosis,treatment and prognosis of the pri-mary bronchial and pulmonary amyloidosis.Methods The clinical features of 10 cases of primary bron-chial and pulmonary amyloidosis were summarized retrospectively.Results The most common symptoms of primary bronchial and pulmonary amyloidosis were coughing,sputum,anhelation,and emptysis.Its diag-nosis relied on fiberoptic bronchoscopy,lung puncture biopsy or VATS biopsy.Positive Congo red stain was the characteristic of pathology.Conclusion The primary bronchial and pulmonary amyloidosis is rare in clinical practice.Its diagnosis relies on pathology.There are no specific medicines for treatment so far.Pa-tient with tracheal involvement and diffuse lesions usually has a poor prognosis.