Publication:

Interstitial lung disease as initial presentation of ANCA associated vasculitis

Interstitial lung disease (ILD) can occur in relation to many autoimmune processes. Occasionally, ILD may also occur in the presence of anti-neutrophil cytoplasmic antibodies (ANCA). In this paper, we report the case of a 60-year-old female with a long-standing history of ILD who later developed anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and glomerulonephritis. Her clinical progression, where ILD emerged prior to systemic manifestations of AAV, supports the hypothesis that rarely ILD may be an early manifestation of ANCA-associated vasculitis. Additionally, we reviewed existing literature examining the connection between ILD and AAV. The published data suggest that ILD may precede ANCA-associated vasculitis, reinforcing the importance of a multidisciplinary approach for optimal management. Further research is necessary to further understand the underlying mechanisms linking ILD and AAV and to develop evidence-based treatment guidelines.