Strobel E S, Bonnet R B, Werner P, Schaefer H E, Peter H H
Department of Medicine, Klinikum der Albert-Ludwigs-Universität Freiburg, Germany.
Clin Rheumatol. 1998;17(3):246-9. doi: 10.1007/BF01451058.
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.
一名患有原发性胆汁性肝硬化6年的55岁女性，急性起病，出现发热、呼吸困难、双下肺野湿啰音，以及弥漫性间质和双肺基底段斑片状肺实变影。排除感染性病因后，进行了开胸肺活检，结果显示有两个组织病理学特征：（1）闭塞性细支气管炎并机化性肺炎；（2）淋巴细胞-组织细胞性间质性肺炎和破坏性细支气管炎。对皮质类固醇和硫唑嘌呤的治疗反应呈双峰模式，最初出现的症状立即缓解，残留的气体交换缺陷则在后期得到缓解。
