Abstract: Objective Anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis （AAV）maintenance hemodialysis recurrent pulmonary hemorrhage is a rare clinical disease. This study aims to improve the understanding of this disease. MethodsThe clinical data of a patient with ANCA-associated vasculitis who suffered from recurrent pulmonary hemorrhage after maintenance hemodialysis were analyzed， and the clinical features，diagnostic points，and treatment plans were discussed in combination with the literature.ResultsOn December 3，2016，the patient was diagnosed with ANCA-related vasculitis in the external hospital and entered maintenance hemodialysis after active treatment failed. On October 7， 2018， hemoptysis and anuria occurred. After admission， the anti-MPO antibody was positive（+） and the anti-GBM antibody was negative（-）. Chest CT showed pulmonary hemorrhage. It was considered to diagnose recurrent diffuse alveolar hemorrhage in ANCA-associated vasculitis maintenance hemodialysis. After antiinflammation （methylprednisolone + cyclophosphamide），hemodialysis，anti-infection， and other treatments， the symptoms improved. ConclusionThe recurrent pulmonary hemorrhage in maintenance hemodialysis patients with ANCA-associated vasculitis is mainly manifested by hemoptysis. Chest CT shows diffuse alveolar hemorrhage. If necessary，bronchoscopic alveolar lavage should be performed to make a clear diagnosis， and a high-dose hormone pulse combined with cyclophosphamide or rituximab should be given，and plasma exchange should be performed as appropriate.

Key words: anti-neutrophil cytoplasmic antibody associated vasculitis, hemodialysis, diffuse alveolar hemorrhage