CAJ | 학술논문

目的：探讨原发性支气管肺淀粉样变的临床表现、影像学特征和治疗措施。方法回顾分析我院1994-2012年收治的9例原发性支气管肺淀粉样变患者的临床资料并结合文献进行分析。结果9例中男6例，女3例，平均年龄54岁。肺实质结节型和气管支气管淀粉样变主要表现为咳嗽、咳痰，可伴咯血或无症状。肺间质弥漫型主要表现为胸痛、痰中带血。肺实质结节型胸部CT均表现为肺部结节影，气管支气管淀粉样变表现为气管、支气管管壁增厚，可伴有钙化，肺间质弥漫性淀粉样变表现为双肺弥漫分布小结节影、斑片影。肺实质结节型通常预后较好。气管支气管型病变者常反复感染加重，需间断气管镜下介入治疗，抗炎、止咳化痰、平喘等综合治疗有一定疗效。呈肺间质弥漫型病变者预后较差。结论原发性支气管肺淀粉样变临床和影像学表现无特异性，确诊依据病理学检查，抗炎等对症治疗可以缓解症状。
목적：탐토원발성지기관폐정분양변적림상표현、영상학특정화치료조시。방법회고분석아원1994-2012년수치적9례원발성지기관폐정분양변환자적림상자료병결합문헌진행분석。결과9례중남6례，녀3례，평균년령54세。폐실질결절형화기관지기관정분양변주요표현위해수、해담，가반각혈혹무증상。폐간질미만형주요표현위흉통、담중대혈。폐실질결절형흉부CT균표현위폐부결절영，기관지기관정분양변표현위기관、지기관관벽증후，가반유개화，폐간질미만성정분양변표현위쌍폐미만분포소결절영、반편영。폐실질결절형통상예후교호。기관지기관형병변자상반복감염가중，수간단기관경하개입치료，항염、지해화담、평천등종합치료유일정료효。정폐간질미만형병변자예후교차。결론원발성지기관폐정분양변림상화영상학표현무특이성，학진의거병이학검사，항염등대증치료가이완해증상。
Objective To study the clinical manifestations, imaging features and treatment of primary bronchial and pulmonary amyloidosis. Methods Clinical data about 9 patients with primary bronchial and pulmonary amyloidosis admitted to our hospital from 1994 to 2012 were retrospectively analyzed with its related literature reviewed. Results The average age of the 9 patients (6 males and 3 females) with primary bronchial and pulmonary amyloidosis included in this study was 54 years. The main clinical manifestations in patients with pulmonary nodular parenchymal and tracheobronchial amyloidosis were cough, expectoration and hemoptysis. The clinical manifestations in patients with diffuse pulmonary interstitial amyloidosis were chest pain and hemoptysis. Chest CT showed nodular shadows in parenchymal amyloidosis patients, thickened tracheobronchial wall or calcification in tracheobronchial amyloidosis patients, and nodular and patching shadows in diffuse pulmonary interstitial parenchymal amyloidosis patients. The prognosis of pulmonary nodular parenchymal amyloidosis patients was usually good. Tracheobronchial amyloidosis usually exacerbated due to repeated infections and required bronchoscopic interventional therapy, anti-inflammation therapy, and cough-suppressing or phlegm-resolving therapy. The prognosis of diffuse pulmonary interstitial amyloidosis patients was usually poor. Conclusion Primary bronchial and pulmonary amyloidosis has no specific clinical and imaging manifestations, and is diagnosed according to its pathology. Expectant treatments such as anti-inflammation therapy can alleviate its symptoms.