Department of Medicine, Thoracic Service, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
Department of Pathology, Thoracic Service, University of British Columbia, Vancouver, BC, Canada.
Chest. 2021 Jun;159(6):e365-e370. doi: 10.1016/j.chest.2021.01.011.
Pleuroparenchymal fibroelastosis (PPFE) is a progressive and frequently fatal interstitial lung disease that involves the upper lobes. Although its cause remains unknown, the histopathologic evidence underlying PPFE bears striking resemblance to that of the pulmonary apical cap (PAC), a relatively common and benign entity. We describe the case of a patient with PAC that evolved into distinctly asymmetric PPFE over 6 years after unilateral surgical lung injury. Given the histologic similarity between these two conditions, we propose that these two entities underlie common biologic pathways of abnormal response to lung injury, with the presence of a PAC increasing susceptibility to the development of PPFE in the face of ongoing inflammatory insults. This case describes the histopathologic evolution of PAC to PPFE before and after an inciting injury.
胸膜肺弹力纤维增生症（PPFE）是一种进行性且常致命的间质性肺疾病，累及肺上叶。尽管其病因仍不清楚，但 PPFE 的组织病理学证据与肺尖帽（PAC）非常相似，后者是一种相对常见且良性的实体。我们描述了一例 PAC 患者的病例，该患者在单侧肺损伤 6 年后进展为明显不对称的 PPFE。鉴于这两种情况的组织学相似性，我们提出这两种实体可能存在共同的生物学途径，即对肺损伤的异常反应，PAC 的存在增加了在持续炎症刺激下发生 PPFE 的易感性。该病例描述了在激发性损伤前后 PAC 向 PPFE 的组织病理学演变。
