There is no uniform classification and diagnostic criteria for ANCA-associated vasculitis, and the diagnosis needs to be made by combining clinical manifestations, serum ANCA tests, characteristic pathological changes and imaging examinations. ANCA-associated vasculitis is an autoimmune disease. The cause of this disease is unknown, and genetic factors, infections, especially bacterial infections, are closely related to the onset of the disease. Patients may present with a variety of clinical manifestations, such as elevated body temperature, cough, hemoptysis, anemia, rash, fever, and increased blood sedimentation, depending on the individual. Currently, there is no standardized diagnostic criteria for ANCA-associated vasculitis, and the diagnosis needs to be made by combining clinical manifestations, serum ANCA tests, characteristic pathological changes and imaging tests. As this disease is relatively complex, it is recommended that patients with ANCA-associated vasculitis should go to the rheumatology and immunology departments of regular hospitals and follow the doctor’s instructions for standardized diagnosis and treatment.