PDF(Pubmed)
Abstract：
While idiopathic pulmonary fibrosis (IPF) is the most common type of fibrotic lung disease, there are numerous other causes of pulmonary fibrosis that are often characterized by lung injury and inflammation. Although often gradually progressive and responsive to immune modulation, some cases may progress rapidly with reduced survival rates (similar to IPF) and with imaging features that overlap with IPF, including usual interstitial pneumonia (UIP)-pattern disease characterized by peripheral and basilar predominant reticulation, honeycombing, and traction bronchiectasis or bronchiolectasis. Recently, the term progressive pulmonary fibrosis has been used to describe non-IPF lung disease that over the course of a year demonstrates clinical, physiologic, and/or radiologic progression and may be treated with antifibrotic therapy. As such, appropriate categorization of the patient with fibrosis has implications for therapy and prognosis and may be facilitated by considering the following categories: (a) radiologic UIP pattern and IPF diagnosis, (b) radiologic UIP pattern and non-IPF diagnosis, and (c) radiologic non-UIP pattern and non-IPF diagnosis. By noting increasing fibrosis, the radiologist contributes to the selection of patients in which therapy with antifibrotics can improve survival. As the radiologist may be first to identify developing fibrosis and overall progression, this article reviews imaging features of pulmonary fibrosis and their significance in non-IPF-pattern fibrosis, progressive pulmonary fibrosis, and implications for therapy. Keywords: Idiopathic Pulmonary Fibrosis, Progressive Pulmonary Fibrosis, Thin-Section CT, Usual Interstitial Pneumonia © RSNA, 2024.
摘要：
特发性肺纤维化（IPF）是最常见的纤维化肺病，肺纤维化还有许多其他原因，通常以肺损伤和炎症为特征。虽然通常是逐渐进展和响应免疫调节，一些病例可能进展迅速，生存率降低（与IPF相似），影像学特征与IPF重叠，包括常见的间质性肺炎（UIP）型疾病，其特征是周围和基底动脉占优势的网状化，蜂窝,牵引支气管扩张或支气管扩张。最近,术语进行性肺纤维化已被用来描述非IPF肺病，在一年的过程中证明临床，生理,和/或放射学进展，可以用抗纤维化疗法治疗。因此，纤维化患者的适当分类对治疗和预后有影响，并且可以通过考虑以下类别来促进：（a）放射学UIP模式和IPF诊断，（b）放射学UIP模式和非IPF诊断，和（c）放射学非UIP模式和非IPF诊断。通过注意到纤维化的增加，放射科医师有助于选择抗纤维化药物治疗可以提高生存率的患者。由于放射科医生可能首先确定正在发展的纤维化和整体进展，本文综述了肺纤维化的影像学特征及其在非IPF型纤维化中的意义，进行性肺纤维化,以及对治疗的影响。关键词：特发性肺纤维化，进行性肺纤维化,薄截面CT,通常的间质性肺炎©RSNA，2024.
