Case 1: A 78-year-old woman was diagnosed as idiopathic pulmonary fibrosis (IPF) at 62 years old and received medical follow-up. She was admitted to the hospital because of acute exacerbation of IPF as a result of viral infection. She passed away before need of intensive care. Histopathological findings of the specimen obtained by lung autopsy showed advanced usual interstitial pneumonia (UIP). Case 2: Bronchoscopy was performed on a 68-year-old woman for patchy ground-glass opacities. A lung biopsy specimen shows lymphocytic inflammation within the interstitium alveolar wall thickening. She was detected having renal insufficiency with high P-ANCA three years after the bronchoscopy. Renal biopsy revealed necrotizing crescentic glomerulonephritis. Finally, she was diagnosed as ANCA-associated vasculitis. Case 3. A 63-year-old man visited the hospital because of persistent cough and dyspnea. He underwent performance of video-assisted thoracic surgery and was diagnosed as UIP. Familial interstitial pneumonia (FIP) is estimated to be 0.5-2.0% of idiopathic interstitial pneumonia. To the best of our knowledge, this is the first report of FIP including ANCA-associated vasculitis and IPF.