A large European multicentre study involving 162 patients provides important new insights into interstitial lung disease (ILD) associated with ANCA antibodies. The findings highlight that lung fibrosis is a serious and often under-recognised complication within the ANCA disease spectrum. One of the key messages emerging from this study is that patients with ANCA-associated ILD should ideally be managed by a multidisciplinary team. Although kidney involvement was common, it did not independently predict mortality. This suggests that lung disease severity at baseline may be the main driver of long-term outcomes in this population.