Centre for Heart and Lung Innovation, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada; Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Department of Internal Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, Yale School of Medicine, New Haven, Connecticut; Department of Chronic Disease, Metabolism and Aging, Laboratory of Respiratory Diseases, KU Leuven, Leuven, Belgium.
Am J Pathol. 2020 Dec;190(12):2427-2435. doi: 10.1016/j.ajpath.2020.09.001. Epub 2020 Sep 11.
Idiopathic pulmonary fibrosis (IPF) is a fibrotic disease with the histology of usual interstitial pneumonia (UIP). Although the pathologist's visual inspection is central in histologic assessments, three-dimensional microcomputed tomography (microCT) assessment may complement the pathologist's scoring. We examined associations between the histopathologic features of UIP and IPF in explanted lungs and quantitative microCT measurements, including alveolar surface density, total lung volume taken up by tissue (%), and terminal bronchiolar number. Sixty frozen samples from 10 air-inflated explanted lungs with severe IPF and 36 samples from 6 donor control lungs were scanned with microCT and processed for histologic analysis. An experienced pathologist scored three major UIP criteria (patchy fibrosis, honeycomb, and fibroblastic foci), five additional pathologic changes, and immunohistochemical staining for CD68-, CD4-, CD8-, and CD79a-positive cells, graded on a 0 to 3+ scale. The alveolar surface density and terminal bronchiolar number decreased and the tissue percentage increased in lungs with IPF compared with controls. In lungs with IPF, lower alveolar surface density and higher tissue percentage were correlated with greater scores of patchy fibrosis, fibroblastic foci, honeycomb, CD79a-positive cells, and lymphoid follicles. A decreased number of terminal bronchioles was correlated with honeycomb score but not with the other scores. The three-dimensional microCT measurements reflect the pathological UIP and IPF criteria and suggest that the reduction in the terminal bronchioles may be associated with honeycomb cyst formation.
特发性肺纤维化（IPF）是一种纤维化疾病，其组织学表现为寻常型间质性肺炎（UIP）。尽管病理学家的肉眼观察在组织学评估中至关重要，但三维微计算机断层扫描（microCT）评估可能可以补充病理学家的评分。我们研究了 UIP 的组织病理学特征与肺纤维化在移植肺中的关系，以及定量 microCT 测量值，包括肺泡表面密度、组织占据的总肺体积（%）和终末细支气管数量。对 10 个充气严重 IPF 移植肺的 60 个冷冻样本和 6 个供体对照肺的 36 个样本进行了 microCT 扫描和组织学分析。一位经验丰富的病理学家对三种主要 UIP 标准（斑片状纤维化、蜂窝和纤维母细胞灶）、五种额外的病理改变以及 CD68、CD4、CD8 和 CD79a 阳性细胞的免疫组织化学染色进行了评分，评分为 0 到 3+。与对照组相比，IPF 肺中的肺泡表面密度降低，终末细支气管数量减少，组织百分比增加。在 IPF 肺中，较低的肺泡表面密度和较高的组织百分比与斑片状纤维化、纤维母细胞灶、蜂窝、CD79a 阳性细胞和淋巴滤泡评分较高相关。终末细支气管数量减少与蜂窝评分相关，但与其他评分无关。三维 microCT 测量值反映了病理性 UIP 和 IPF 标准，并表明终末细支气管的减少可能与蜂窝状囊肿的形成有关。
