Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation
Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation
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肺移植患者特发性和继发性间质性肺炎伴胸膜实质弹力纤维增生症的临床、影像学和病理学特征
DOI：
10.1111/his.14595
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发表时间：
2022
期刊：
影响因子：
6.4
通讯作者：
Handa Tomohiro 他
中科院分区：
文献类型：
--
作者：
Ikegami Naoya;Nakajima Naoki;Yoshizawa Akihiko;Handa Tomohiro 他
目的特发性胸膜实质纤维弹力纤维增生症(PPFE)是一种罕见的特发性间质性肺炎，继发性间质性肺炎也可见病理性PPFE。本研究旨在评价与放射性PPFE样病变相关的病理表现以及病理性PPFE患者的临床和形态学特征。方法与结果回顾59例肺移植受者移植肺的病理资料。在14例特发性疾病和12例继发性疾病患者中发现了病理性PPFE病变。病理性PPFE与既往气胸、上肺叶血容量减少和胸部平坦有关。特发性肺纤维化与继发性肺纤维化的临床、影像和病理表现相似，而成纤维细胞病变在特发性肺纤维化中更常见，继发性肺泡间隔增厚伴弹力纤维增厚或纤维化的发生率更高。特发性和继发性病理性肺纤维化患者移植后生存率差异无统计学意义(LOG-RANK；P= 0.5 7)，而特发性肺纤维化患者与特发性肺纤维化患者移植后生存率相似(LOG-RANK；P= 0.6 2)。特征性的临床特征提示病理性PPFE的存在，特发性和继发性病理性PPFE相似，除了特发性的成纤维细胞灶和继发性的肺泡间隔增厚伴弹性增厚或纤维化。病理性PPFE患者移植后的预后与IPF患者相似。
AimsIdiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, and pathological PPFE is also observed in patients with secondary interstitial pneumonia. This study aimed to evaluate the pathological findings associated with radiological PPFE‐like lesions and the clinical and morphological features of patients with pathological PPFE.Methods and resultsWe retrospectively reviewed the pathology of the explanted lungs from 59 lung transplant recipients with radiological PPFE‐like lesions. Pathological PPFE lesions were identified in 14 patients with idiopathic disease and in 12 patients with secondary disease. Pathological PPFE was associated with previous pneumothorax, volume loss in the upper lobes, and a flattened chest. Patients with idiopathic disease and those with secondary disease with pathological PPFE had similar clinical, radiological and pathological findings, whereas fibroblastic foci were more common in patients with idiopathic disease, and patients with secondary disease more frequently showed alveolar septal thickening with elastosis or fibrosis. Post‐transplantation survival did not differ between patients with idiopathic and secondary disease with pathological PPFE (log‐rank;P= 0.57) and was similar between patients with idiopathic disease with pathological PPFE and those with idiopathic pulmonary fibrosis (IPF) (log‐rank;P= 0.62).ConclusionsNot all patients with interstitial pneumonia with radiological PPFE‐like lesions have pathological PPFE. Characteristic clinical features can suggest the presence of pathological PPFE, and idiopathic and secondary cases with pathological PPFE are similar except for fibroblastic foci in idiopathic cases and alveolar septal thickening with elastosis or fibrosis in secondary cases. Patients with pathological PPFE have a similar prognosis to those with IPF after transplantation.
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