Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias
Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias
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DOI：
10.1097/01.pas.0000213341.70852.9d
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发表时间：
2007-02-01
影响因子：
5.6
通讯作者：
Wright, Joanne L.
中科院分区：
文献类型：
--
作者：
Churg, Andrew;Mueller, Nestor L.;Wright, Joanne L.
普通型间质性肺炎（UIP）的急性加重是一种UIP患者，偶尔也有其他形式的纤维化间质性肺病，出现快速呼吸衰竭，伴有广泛的放射性浸润。这种情况的病理特征在文献中定义不清，结局也不清楚。我们报告了12例这样的患者，9例有潜在的UIP，2例有潜在的纤维化非特异性间质性肺炎，1例有潜在的慢性过敏性肺炎，他们接受了手术肺活检诊断。11例病例的高分辨率计算机断层扫描数据显示存在广泛的双侧毛玻璃样阴影，有时伴有局灶性实变，叠加在基础纤维化上。观察到三种急性肺损伤的显微镜模式：弥漫性肺泡损伤（DAD）、机化性肺炎（OP）和叠加在基础纤维化上的大量非常大的成纤维细胞病灶模式。活检后，所有患者均接受类固醇治疗，在某些情况下伴有环磷酰胺或硫唑嘌呤。10例患者在急性发作后存活，出院时存活时间为1至11个月;在这些病例中，6例显示OP或OP+广泛成纤维细胞灶模式; 2例仅显示广泛成纤维细胞灶模式; 2例显示DAD模式。2例死亡患者均患有组织学DAD。我们的结论是，急性加重的UIP和其他纤维化肺疾病产生了各种病理模式的活检，并与OP或广泛的成纤维细胞病灶的急性模式的患者似乎比那些与DAD做得更好。我们的数据还表明，生存率（急性发作）可能比文献所示的要好。
Acute exacerbation of usual interstitial pneumonia (UIP) is a condition in which patients with UIP, and occasionally other forms of fibrotic interstitial lung disease., develop rapid respiratory failure, accompanied by extensive radiologic infiltrates. The pathologic features of this condition are ill-defined in the literature and the outcome is unclear. We report 12 such patients, 9 with underlying UIP, 2 with underlying fibrotic nonspecific interstitial pneumonia, and I with underlying chronic hypersensitivity pneumonitis, who underwent surgical lung biopsy for diagnosis. High-resolution computed tomography data were available in 11 cases and showed the presence of extensive bilateral ground-glass opacities, sometimes accompanied by focal consolidation, superimposed on underlying fibrosis. Three microscopic patterns of acute lung injury were seen: diffuse alveolar damage (DAD), organizing pneumonia (OP), and a pattern of numerous very large fibroblast foci superimposed on underlying fibrosis. After the biopsy, all patients were treated with steroids, in some instances accompanied by cyclophosphamide or azathioprine. Ten patients survived the acute episode and were discharged with survival times of 1 to 11 months; of these cases, 6 showed a pattern of OP or OP plus extensive fibroblast foci; 2 a pattern of extensive fibroblast foci only; and 2 a pattern of DAD. Both patients who died had histologic DAD. We conclude that acute exacerbation of UIP and other fibrotic lung diseases produces a variety of pathologic patterns on biopsy, and that patients with OP or extensive fibroblast foci as the acute pattern seem to do better than those with DAD. Our data also imply that survival (of the acute episode) may be better than the literature suggests.