RT Journal Article SR Electronic T1 Antineutrophil cytoplasmic antibody-associated interstitial lung disease: a review JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 210123 DO 10.1183/16000617.0123-2021 VO 30 IS 162 A1 Suha Kadura A1 Ganesh Raghu YR 2021 UL http://err.ersjournals.com/content/30/162/210123.abstract AB Over the past three decades, an increasing number of publications have reported the association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA) or ANCA-associated vasculitis (AAV). With this increased awareness, we have reviewed the literature to date and provide an update in this narrative review. The vast majority of cases of ILD have been shown to be in the setting of positive anti-myeloperoxidase antibody and can be present in up to 45% of patients of microscopic polyangiitis, though cases of ILD associated with proteinase 3 ANCA have rarely been reported. Pulmonary fibrosis and ANCA positivity can occur with or without systemic involvement. The pathogenetic mechanisms establishing the relationship between ANCA and the development of pulmonary fibrosis remain unclear. Histologic and radiographic features of ANCA-ILD most commonly reveal usual interstitial pneumonia or non-specific interstitial pneumonia patterns, though other atypical features such as bronchiolitis have been described. ILD in the setting of AAV has been associated with worse outcomes, and thus early identification and treatment in these patients is appropriate. We advocate that ANCA antibody testing be performed as a baseline evaluation in patients presenting with idiopathic interstitial pneumonia. Suggested treatment of ANCA-ILD includes immunosuppression and/or antifibrotic agents, though supporting data and clinical trials to substantiate use of these therapies are needed.Over the last three decades, an increasing number of publications have reported the association between interstitial lung disease and anti-neutrophil cytoplasmic antibody (ANCA) or ANCA-associated vasculitis. With this increased awareness, we have reviewed the literature to date and provide an update in this narrative review. https://bit.ly/3A8SGHZ