原发性气管支气管淀粉样变临床分析

目的 探讨原发性气管支气管淀粉样变的临床特征、诊疗方法及预后.方法 对本院1例及1989至2012年中文文献报道的50例原发性气管支气管淀粉样变病例进行回顾性总结,分析其基本资料、临床表现、影像学表现、支气管镜下表现及治疗情况等资料.结果 本院患者因“气急”入院,行胸部CT、支气管镜以及肺功能检查,以支气管镜活检获取标本确诊,经环磷酰胺治疗后好转.文献报道的50例患者主要临床表现为咳嗽(96％,48/50)、气急(70％,35/50)、咳痰(66％,33/50)、发热(24％,12/50)及咯血(22％,11/50)等；胸片检查以肺部纹理增粗(45.5％,15/33)最为常见,胸部CT影像学表现以气管壁增厚、管腔狭窄(76.2％,32/42)最为常见；最常见的支气管镜下表现为气管壁增厚、管腔狭窄(40.8％,20/49),其次为黏膜充血、水肿(32.7％,16/49);肺功能检查以阻塞性通气功能障碍为最主要表现.3例伴有并发症,24例误诊为其他肺部疾病.最终47例(94％)患者经支气管镜活检确诊,2例经CT引导下肺穿刺活检确诊,1例经手术后病理确诊.50例患者中42例接受药物、支气管镜下介入、胸部外照射及手术等治疗,病情均好转.结论 原发性气管支气管淀粉样变是一种临床少见病,易出现误诊,确诊需依靠病理学检查,目前尚无特异性治疗方法.

Clinical analysis of primary tracheobronchial amyloidosis

Objective To discuss the clinical features,diagnosis,treatment and prognosis of primary tracheobronchial amyloidosis.Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital.The clinical information,symptoms,imaging findings,endoscopic manifestations and treatment were analyzed.Results The patient in our hospital was admitted due to "short breath".Chest CT,bronchoscopy and pulmonary function examination were done;bronchoscopy biopsy specimen confirmed the diagnosis of PTA;and the patients responded to cyclophosphamide treatment.The main clinical symptoms of the 50 patients included cough(96%,48/50),short breath(70%,35/50),sputum(66%,33/50),fever(24%,12/50),and hemoptysis(22%,11/50).Thickening of lung markings(45.5%,15/33)was the most common chest X-ray finding;airway wall thickening and stenosis(76.2%,32/42) was the most common CT manifestation;and the most common endoscopic finding was also airway wall thickening and stenosis(40.8%,20/49),followed by mucosal congestion and edema(32.7%,16/49).Obstructive ventilatory dysfunction was found to be the major problem during pulmonary function tests.The diagnoses in 47(94%) patients were confirmed by bronchoscopic biopsy.Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases.Forty-two of the 50 patients received drug treatment,bronchoscopic interventional therapy,external beam radiation therapy and surgical treatment,and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed.Correct diagnose needs pathological examination and currently there is no specific treatment for it.