肺淀粉样变性的临床和CT表现

Clinical and CT Diagnosis of Pulmonary Amyloidosis

目的探讨肺淀粉样变性（PA）的临床和CT表现,以提高对该病的认识和鉴别诊断能力。方法回顾性分析经病理证实的16例PA患者的CT表现及临床资料,并结合文献进行复习。结果 16例患者中男10例,女6例;年龄39-84岁,平均（55.68±10.07）岁。主要临床表现：活动后气短（11/16）、咳嗽（9/16）、咳痰（3/16）、胸闷（2/16）、咯血（2/16）。CT表现分三型：气管支气管型、肺实质型、肺间质型。气管支气管型6例,表现为气管支气管壁增厚、狭窄,其中4例伴钙化;肺实质型3例,其中2例多发结节,1例单发结节;肺间质型7例,均可见双肺小叶间隔增厚,其中2例表现为双肺弥漫性间质性病变。结论 PA临床少见,其临床和CT表现具有相对特征性,但确诊仍需依赖病理。CT对PA分型及指导临床制定治疗方案具有重要价值。

Objective To investigate the clinical and imaging features of pulmonary amyloidosis（ PA）,in order to improve the diagnosis and differential diagnosis of the disease. Methods The clinical and CT findings of 16 cases with pulmonary amyloidosis proved pathologically were retrospectively analyzed,. and combined with literature review. Results10 males and 6 females,aged from 39 to 84 years,with an average age of 55. 68 ± 10. 07 years. The main clinical manifestations： shortness of breath（ 11/16）,cough（ 9/16）,expectoration（ 3/16）,chest pain（ 2/16）,hemoptysis（ 2/16）. CT expression was divided into three types： trachea and bronchus type,pulmonary parenchyma type,pulmonary interstitial type. 6 cases of tracheobronchial type,showed airway wall thickening,stenosis,4 cases with calcification; 3 cases of pulmonary nodules,including 2 cases of multiple nodules,1 case of solitary nodules; 7 cases of interstitial lung showed double interlobular septal thickening,and 2 of them showed diffuse interstitial lung disease. Conclusion The progress of pulmonary amyloidosis is slow. The imaging findings with the relative characteristics of CT on pulmonary amyloidosis types can be of important value in guiding the clinical treatment.