Servicio de Radiodiagnóstico, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Servicio de Radiodiagnóstico, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Radiologia (Engl Ed). 2022 Dec;64 Suppl 3:301-307. doi: 10.1016/j.rxeng.2022.12.002.
The term idiopathic pleuroparenchymal fibroelastosis refers to a rare interstitial lung disease that predominantly involves the upper lobes. It has been considered a rare subtype of interstitial lung disease since 2013, when it was included in the joint consensus statement on the diagnosis of interstitial lung diseases published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS). Currently, two distinct types of pleuroparenchymal fibroelastosis are recognized: the idiopathic type for cases in which it has not been possible to establish a specific etiology and a secondary type associated with a variety of different causes. The diagnosis of pleuroparenchymal fibroelastosis must be managed from a combined clinical and radiological perspective. High-resolution computed tomography (HRCT) is the imaging method of choice for the evaluation and diagnosis of pleuroparenchymal fibroelastosis. In many cases, the diagnosis will be based exclusively on the HRCT findings and histologic confirmation will be unnecessary. This article describes the clinical, radiological, and histological characteristics of pleuroparenchymal fibroelastosis, discussing the different associations with this entity and its differential diagnosis.
特发性胸膜肺弹力纤维增生症是一种罕见的间质性肺疾病，主要累及肺上叶。自 2013 年美国胸科学会（ATS）和欧洲呼吸学会（ERS）联合发布间质性肺疾病诊断共识以来，它被认为是一种罕见的间质性肺疾病亚型。目前，已认识到两种不同类型的胸膜肺弹力纤维增生症：特发性类型是指无法确定特定病因的病例，以及继发性类型与多种不同病因相关。胸膜肺弹力纤维增生症的诊断必须从临床和影像学相结合的角度来管理。高分辨率计算机断层扫描（HRCT）是评估和诊断胸膜肺弹力纤维增生症的首选影像学方法。在许多情况下，诊断将完全基于 HRCT 结果，并且不需要组织学证实。本文描述了胸膜肺弹力纤维增生症的临床、放射学和组织学特征，讨论了与该疾病相关的不同关联及其鉴别诊断。
