Interstitial Lung Disease and ANCA-Associated Vasculitis

Presentation and progression of MPO-ANCA interstitial lung disease

“…Lung involvement is a common clinical feature of AAV, often presenting as dry cough, shortness of breath, wheezing, or hemoptysis and including necrotizing granulomatous inflammation, tracheobronchial inflammation, pulmonary capillaritis leading to diffuse alveolar hemorrhage, asthma, and interstitial lung disease (ILD) [ [6] , [7] , [8] ]. The association between AAV, almost exclusively MPO-AAV, and ILD has been known since at least 1990 and it has been increasingly recognized [ [9] , [10] , [11] , [12] , [13] ]. Pulmonary fibrosis may coexist, follow, or even precede the diagnosis of overt AAV and has negative impact on long-term prognosis [ [14] , [15] , [16] ].…”