Abstract: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a class of autoimmune diseases characterized by inflammation of the small vessel wall and fibrinoid necrosis that can involve multiple systems throughout the body. AAV in the Asian region is mainly characterized by microscopic polyangitis (MPA), which most commonly affects the kidneys and lungs. In recent years, it has been discovered that complement such as C5a plays an important role in the pathogenesis of AAV through alternative pathways. Therefore, understanding the relationship between AAV complement and coagulation processes is beneficial for identifying therapeutic targets from the perspective of pathogenesis. This review summerized the research progress on the changes in coagulation and fibrinolysis systems and their interactions with the complement system in the pathogenesis of AAV.