Diagnostic and management controversies in ANCA-associated interstitial lung diseases.
DOI:
https://doi.org/10.52692/1857-0011.2025.3-83.35Keywords:
ANCA vasculitis, Interstitial lung disease, Antifibrotic therapyAbstract
ANCA-associated vasculitides (AAVs) are rare but carry major prognostic significance. Increasing evidence shows that they extend beyond vascular inflammation and frequently involve interstitial lung disease (ILD), most often with a UIP pattern, which is linked to high mortality. The issue is particularly relevant since ILD may precede vasculitis and mimic isolated pulmonary fibrosis, raising the risk of underdiagnosis. Conventional vasculitis therapies have proven ineffective in halting fibrosis and increase infection risk, while antifibrotic agents offer promise but remain insufficiently studied. The absence of clear guidelines and dedicated trials creates clinical uncertainty, making AAV-ILD an emerging field at the crossroads of pulmonology and rheumatology, where urgent research is needed and the potential to improve outcomes is considerable.
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