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克罗恩病中的非肉芽肿性间质性肺病

Nongranulomatous interstitial lung disease in Crohn's disease.

作者信息

Hotermans G, Benard A, Guenanen H, Demarcq-Delerue G, Malart T, Wallaert B

机构信息

Service de Pneumologie et Immunoallergologie, Hôpital Calmette, Lille, France.

出版信息

Eur Respir J. 1996 Feb;9(2):380-2. doi: 10.1183/09031936.96.09020380.

DOI:10.1183/09031936.96.09020380
PMID:8777981
Abstract

Crohn's disease is a granulomatous systemic disorder of unknown aetiology. Obvious pulmonary involvement is exceptional. We report the case of a 33 year old woman treated with mesalazine for Crohn's disease and presenting with dyspnoea. Pulmonary function tests showed a restrictive ventilatory pattern with hypoxaemia on exertion. Chest radiography disclosed an interstitial pattern with ground glass on high resolution computer tomography. Clinical and radiological abnormalities progressed after withdrawal of mesalazine. Corticosteroids led to a partial regression but were stopped because of severe side-effects. Because of worsening of the clinical situation, open lung biopsy was performed and showed a histopathological aspect of nongranulomatous interstitial diffuse lung disease with an inflammatory lymphoid infiltration associated to some mild interstitial collagen fibrosis. Addition of cyclophosphamide to high-dose pulse steroid therapy induced a significant and sustained improvement.

摘要

克罗恩病是一种病因不明的肉芽肿性全身性疾病。明显的肺部受累情况较为罕见。我们报告一例33岁女性病例,该患者因克罗恩病接受美沙拉嗪治疗,出现呼吸困难。肺功能测试显示为限制性通气模式,运动时出现低氧血症。胸部X线检查显示间质改变,高分辨率计算机断层扫描显示磨玻璃影。停用美沙拉嗪后,临床和影像学异常进展。皮质类固醇治疗使病情部分缓解,但因严重副作用而停药。由于临床情况恶化,进行了开胸肺活检,组织病理学显示为非肉芽肿性间质性弥漫性肺病,伴有炎性淋巴细胞浸润及一些轻度间质胶原纤维化。在高剂量脉冲类固醇治疗基础上加用环磷酰胺后,病情得到显著且持续的改善。

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