Bonifazi Martina, Montero M Angeles, Renzoni Elisabetta A
Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy.
Pulmonary Diseases Unit, Department of Internal Medicine, Azienda Ospedali Riuniti, Ancona, Italy.
Curr Pulmonol Rep. 2017;6(1):9-15. doi: 10.1007/s13665-017-0160-5. Epub 2017 Jan 27.
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current evidence on IPPFE, in terms of clinical features and potential treatments.
Overall, there is increasing awareness of PPFE in association with a separate ILD pattern. Although an agreed consensus on diagnosis has yet to be defined, a list of radiological and histopathological criteria has been proposed. Due to the unfavorable risk-benefit profile of surgical lung biopsy in a significant proportion of patients, a potential role for transbronchial lung cryobiopsy has been suggested. At present, lung transplantation remains the only curative option.
The increasing awareness of this condition among specialists has led to more frequent identification of IPPFE. Large international studies are needed to better characterize pathogenesis and pheno/endotypes of disease, a key step towards the development of effective treatments.
特发性胸膜肺实质纤维弹性组织增生症（IPPFE）是一种罕见的肺纤维化疾病，主要累及脏层胸膜和胸膜下实质，以上叶受累为主，在最新的特发性间质性肺炎（IIP）国际多学科分类中被列为一种独特的临床病理实体。我们旨在总结目前关于IPPFE在临床特征和潜在治疗方面的证据。
总体而言，人们越来越认识到PPFE与一种单独的间质性肺疾病模式相关。尽管尚未就诊断达成共识，但已提出了一系列放射学和组织病理学标准。由于在相当一部分患者中手术肺活检的风险效益比不佳，有人提出了经支气管肺冷冻活检的潜在作用。目前，肺移植仍然是唯一的治愈选择。
专家们对这种疾病的认识不断提高，导致IPPFE的诊断更为频繁。需要开展大型国际研究，以更好地描述该疾病的发病机制和表型/内型，这是开发有效治疗方法的关键一步。
