Bronchiolitis obliterans organising pneumonia and primary biliary cirrhosis-like lung involvement in a patient with primary biliary cirrhosis
Bronchiolitis obliterans organising pneumonia and primary biliary cirrhosis-like lung involvement in a patient with primary biliary cirrhosis
复制标题
原发性胆汁性肝硬化患者的闭塞性细支气管炎伴机化性肺炎和原发性胆汁性肝硬化样肺部受累
作者：
E. ;R. B. Bonnet;P. Werner;H. ;H. H. Peter
55岁女性，原发性胆汁性肝硬化6年病史，急性起病发热，呼吸困难，双肺下野有裂纹，弥漫性间质性和双基底片状肺混浊。在排除感染性病因后，行开放性肺活检，发现两种组织病理学特征：(1)闭塞性细支气管炎组织性肺炎；(2)淋巴组织细胞间质性肺炎和破坏性细支气管炎。皮质类固醇和硫唑嘌呤的治疗反应遵循双峰模式，她的初始症状立即消退，残余气体交换缺陷的消退较晚。
A 55-year-old woman with a 6-year history of primary biliary cirrhosis presented with an acute onset of fever, dyspnoea, crackles over both lower lung fields, and diffuse interstitial and bibasilar patchy pulmonary opacities. After exclusion of an infectious aetiology, an open lung biopsy was performed which revealed two histopathological features: (1) bronchiolitis obliterans organising pneumonia and (2) lympho-histiocytic interstitial pneumonitis and destructive bronchiolitis. Treatment response to corticosteroids and azathioprine followed a bimodal pattern with immediate resolution of her initial presenting symptoms and late resolution of residual gas exchange defects.