Maillet T, Goletto T, Beltramo G, et al. - Sixty-two cases (89% MPO-antineutrophil cytoplasm antibody [ANCA]+) were involved in order to determine the outcomes of patients with interstitial lung disease (ILD) with related ANCA-associated vasculitis (AAV)(AAV–ILD). ILD was diagnosed prior to or concurrently with AAV. Cases in comparison with 124 controls less often had systemic vasculitis symptoms. Multivariate analyses engaged age > 65 years, alveolar hemorrhage and usual interstitial pneumonia (UIP), although, not immunosuppressant use, as factors independently related to shorter survival. Hence, only UIP was associated with a weaker prognosis for AAV–ILD patients. Moreover, immunosuppressants did not enhance the AAV–ILD prognosis. However, in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents could be helpful and should be evaluated in patients with AAV–ILD with a UIP pattern.