Chronic hypersensitivity pneumonitis

Chronic hypersensitivity pneumonitis
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DOI:
10.1097/01.pas.0000184806.38037.3c
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发表时间:
2006-02-01
影响因子:
5.6
通讯作者:
Wright, JL
Wright, JL
中科院分区:
医学1区
文献类型:
--
作者:
Churg, A;Muller, NL;Wright, JL

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过敏性肺炎(HP)传统上根据临床分为急性、亚急性和慢性期。大多数活检标本来自亚急性期的患者,其中存在相对轻度的,通常是细支气管周围的慢性间质炎性浸润,大多数情况下伴有形成不良的间质肉芽肿或孤立的巨细胞。然而,在慢性,即纤维化阶段的病理特征,在文献中定义不清。认识这些特征很重要,因为HP的慢性阶段通常与预后不良相关。我们回顾了13例慢性HE的资料,一般发生在很长一段时间内的致敏剂的暴露。观察到三种纤维化模式:1)以片状模式为主的外周纤维化,伴有结构扭曲和成纤维细胞病灶,在显微镜下类似于普通型间质性肺炎(UIP); 2)相对均匀的线性纤维化,类似于纤维化非特异性间质性肺炎(NSIP);和3)不规则的以支气管周围纤维化为主。在所有病例中,散在的不良肉芽肿,或孤立的间质巨细胞,或有时只有Schaumann小体的存在表明正确的诊断。在7例病例中,还存在典型的亚急性HP区域。高分辨率CT扫描显示不同的模式,从严重的纤维化,在某些情况下,以上区为主,以毛玻璃样阴影为主,周围网状。我们的结论是,在形态学水平,慢性HP可能密切模仿UIP或纤维化NSIP。如果没有明显的亚急性HP区域,则存在孤立的巨细胞、不良形成的肉芽肿或Schaumann小体对正确诊断至关重要,发现细支气管周围纤维化可能有帮助。尽管存在广泛的纤维化,但一些患者对从暴露和类固醇治疗中移除有反应。
Hypersensitivity pneumonitis (HP) is traditionally divided on clinical grounds into acute, subacute, and chronic stages. Most biopsy specimens come from patients in the subacute stage, in which there is a relatively mild, usually peribronchiolar, chronic interstitial inflammatory infiltrate, accompanied in most cases by poorly formed interstitial granulomas or isolated giant cells. However, the pathologic features in the chronic, ie, fibrotic stage, are poorly defined in the literature. These features are important to recognize because the chronic stage of HP is often associated with a poor prognosis. We reviewed 13 cases of chronic HE Where information was available, exposures to the sensitizing agent had generally occurred over a long period of time. Three patterns of fibrosis were seen: 1) predominantly peripheral fibrosis in a patchy pattern with architectural distortion and fibroblast foci resembling, microscopically, usual interstitial pneumonia (UIP); 2) relatively homogeneous linear fibrosis resembling fibrotic nonspecific interstitial pneumonia (NSIP); and 3) irregular predominantly peribronchiolar fibrosis. In some instances, mixtures of the UIP-like and peribronchiolar patterns were found. In all cases, the presence of scattered poorly formed granulomas, or isolated interstitial giant cells, or sometimes only Schaumann bodies indicated the correct diagnosis. In 7 cases, areas of typical subacute HP were present as well. High-resolution CT scans showed variable patterns ranging from severe fibrosis, in some instances with an upper zone predominance, to predominantly ground glass opacities with peripheral reticulation. We conclude that, at the level of morphology, chronic HP may closely mimic UIP or fibrotic NSIP. If no areas of subacute HP are evident, the presence of isolated giant cells, poorly formed granulomas, or Schaumann bodies is crucial to arriving at the correct diagnosis, and the finding of peribronchiolar fibrosis may be helpful. Despite the presence of extensive fibrosis, some patients responded to removal from exposure and steroid therapy.