Interstitial Lung Disease in ANCA-Associated Vasculitis: Pathogenic Considerations and Impact for Patients ’ Outcomes

AbstractPurpose of ReviewThis review provides an update on recent advances in the diagnosis, pathogenesis, clinical presentation, histopathological findings, and treatment approaches for antineutrophil cytoplasmic antibody (ANCA) vasculitis –associated interstitial lung disease (AAV-ILD) with a focus on literature published in the last 3 years.Recent FindingsAlthough there is no validated definition of AAV-ILD, which contributes to some of the heterogeneity seen in study results, there has been an increasing number of publications in recent years on this topic. Most patients with AAV-ILD have MPO-ANCA vasculitis, and this association appears to reduce their 5-year-survival to 60 –66% (Sun et al. BMC Pulm Med 21(1),2021, Maillet et al. J Autoimmun 106,2020). Median age of diagnosis ranges from mid-60  s to mid-70 s (Ando et al. Respir Med 107(4),2013), Kagiyama et al. BMJ Open Respir Res 2(1):1 –9,2015, Hozumi et al. Lung 194(2):235 –42,2016, Liu et al. Chest 156(4):715 –23,2019, Maillet et al. J Autoimmun 106,2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37 –42,2020, Watanabe et al. BMC Pulm Med 19(1),2019). Computed tomography (CT) chest imaging for patients with AAV-ILD often shows a usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) pattern (12 –58% and 13–61%, respectively) (Sun et al. BMC Pulm Med 21(1),2021, Maillet et al. J Autoimmun 106,2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37 –42,...
Source: Current Rheumatology Reports - Category: Rheumatology Source Type: research