University of Kansas School of Medicine, Division of Pulmonary and Critical Care, Kansas City, KS, USA
University of Colorado, Division of Pulmonary Sciences and Critical Care Medicine, Aurora, CO, USA.
Eur Respir J. 2021 May 13;57(5). doi: 10.1183/13993003.02533-2020. Print 2021 May.
Rheumatoid arthritis associated interstitial lung disease (RA-ILD) and idiopathic pulmonary fibrosis (IPF) are distinct diseases; however, they share several clinical, radiographic and genetic features. For instance, usual interstitial pneumonia (UIP), which is an ILD pattern required for a diagnosis of IPF, is also the most common ILD pattern in RA-ILD. The presence of UIP in RA-ILD is a poor prognostic sign with outcomes similar to those seen in IPF. The recent finding of a shared genetic susceptibility between IPF and RA-ILD has sparked additional interest in this relationship. This review outlines these similarities and differences in clinical presentation, appearance and outcomes in RA-ILD and IPF.In addition, this review highlights previous research in molecular biomarkers in both conditions, exploring areas of overlap and distinction. This focus on biomarkers in IPF and RA-ILD aims to highlight potential areas of discovery and clues to a potential shared pathobiology through investigation of novel molecular markers or the repurposing of biomarkers from one condition to the other.The drive to better understand RA-ILD by leveraging our knowledge of IPF is underscored by our divergent treatment paradigms for these conditions and the concern for potential harm. As a result of advancing our understanding of the links between IPF and RA-ILD, current strategies for diagnosis, screening and treatment of ILD may fundamentally change in the coming years. Until then, clinicians face difficult clinical questions regarding the co-management of the articular disease and the ILD in RA.
类风湿关节炎相关间质性肺病(RA-ILD)和特发性肺纤维化(IPF)是两种不同的疾病;然而,它们具有许多相似的临床、影像学和遗传学特征。例如,特发性肺纤维化的诊断需要一种间质性肺病模式,即普通型间质性肺炎(UIP),也是 RA-ILD 中最常见的间质性肺病模式。RA-ILD 中 UIP 的存在是预后不良的标志,其结局与 IPF 相似。最近发现 IPF 和 RA-ILD 之间存在共同的遗传易感性,这引发了人们对这种关系的更多兴趣。本文概述了 RA-ILD 和 IPF 在临床表现、影像学表现和结局方面的这些相似和不同之处。此外,本文还强调了这两种疾病在分子生物标志物方面的先前研究,探讨了重叠和区别的领域。本文重点关注 IPF 和 RA-ILD 中的生物标志物,旨在通过研究新的分子标志物或从一种疾病重新利用生物标志物来探索潜在的共同发病机制,从而突出发现的潜在领域和线索。通过利用我们对特发性肺纤维化的认识来更好地理解类风湿关节炎相关间质性肺病,强调了这一点,因为这两种疾病的治疗模式存在差异,而且存在潜在的危害。随着我们对特发性肺纤维化和类风湿关节炎相关间质性肺病之间联系的理解的提高,ILD 的诊断、筛查和治疗策略可能在未来几年发生根本变化。在那之前,临床医生面临着关于关节疾病和类风湿关节炎中间质性肺病的共同管理的困难临床问题。