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Invasive pulmonary aspergillosis (IPA) has been reported as a severe opportunistic infection in immunocompromised patients without neutropenia or cancer. Patients with antineutrophil cytoplasmic antibody associated vasculitis (AAV) with immunosuppressive treatment are susceptible to IPA, but only few cases were reported in the literature. We retrospectively analyze the clinical characteristics of our patients with IPA in AAV.Hospitalized patients with AAV who developed IPA were selected. Their clinical data were retrospectively reviewed and possible risk factors for development of IPA were investigated.Seven of 157 patients with AAV were identified to have IPA. Two patients were classified as Wegener granulomatosis and 5 as microscopic polyangiitis with a mean age at 68.6 ± 10.9 years. After immunosuppressive therapy, 7 patients developed IPA within 2∼13 weeks. They had 1 or more risk factors increasing susceptibility to Aspergillus. Pre-existing chronic respiratory diseases were found in 5 patients. Despite intensive antifungal therapy, only 3 patients survived. The patients who died were older, with more severe lung injury and lower hemoglobin level.AAV patients with immunosuppressive therapy are susceptible to Aspergillus infection. Monitoring and prophylactic antifungal therapy should be recommended for patients at high risk.