Acute exacerbation of idiopathic pulmonary fibrosis: report of a series
Acute exacerbation of idiopathic pulmonary fibrosis: report of a series
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DOI：
10.1183/09031936.03.00022703
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发表时间：
2003-11-01
影响因子：
24.3
通讯作者：
Poletti, V
中科院分区：
文献类型：
--
作者：
Ambrosini, V;Cancellieri, A;Poletti, V
本研究描述五例无特殊诱因的急性肺纤维化的临床病程，并回顾2001-2002年间经组织学证实为常见间质性肺炎的五例病例。报告临床资料、支气管肺泡灌洗(BAL)结果、高分辨率CT和组织学特征。入院时所有病例均表现为低氧血症和呼吸困难，部分病例表现为糖类抗原19.9升高或实验室检查典型的感染，尽管适当的培养均为阴性。总共有四名受试者死亡，只有一人在随访中。所有病例的肺部影像均可见弥漫性磨玻璃样或肺泡状混浊，叠加在网状和线状改变上。BAL上可见明显的中性粒细胞增多和11型反应性细胞增生。组织学检查发现，肺组织学表现与常见的间质性肺炎叠加急性肺损伤的特征，如弥漫性肺泡损伤、伴或不伴透明膜、11型反应性细胞增生和大量成纤维细胞灶。这项研究还强调了支气管肺泡灌洗与开胸肺活检的诊断价值。
This study describes five cases presenting an acute clinical course of pulmonary fibrosis, in the absence of specific precipitating factors.A retrospective chart review of five patients with histologically proved usual interstitial pneumonia was carried out in 2001-2002. Clinical data, bronchoalveolar lavage (BAL) findings, high resolution computed tomography and histological features were reported.On admission all cases presented hypoxemia and dyspnoea, while some showed an increase of carbohydrate antigen 19.9 or laboratory tests typical of infection, although appropriate cultures were all negative. Altogether, four subjects died and only one is on follow-up. A pattern of diffuse ground-glass or alveolar opacification superimposed on reticular and linear findings was evident on lung imaging in all cases. Marked neutrophilia, together with type 11 reactive cells hyperplasia, was detected on BAL.Histological findings, from open lung biopsy or autopsy, showed all the aspects of usual interstitial pnemonia with superimposed features of acute lung injury, such as diffuse alveolar damage, with or without hyaline membranes, type 11 reactive cells hyperplasia and numerous fibroblastic foci. This study also underlines the diagnostic value of bronchoalveolar lavage versus open lung biopsy.