Neutrophil anticytoplasmic antibodies 1:10 are commonly seen in Wegener’s granulomatosis, but can also be seen in vasculitis polyarteritis. However, antineutrophil anticytoplasmic antibodies alone are not sufficient to make a definitive diagnosis of the disease, which should be made in conjunction with the patient’s medical history, signs, symptoms, and other laboratory findings. Neutrophil anticytoplasmic antibodies are one of the models of antineutrophil antibodies, and the antigen is mainly protease-3. If antineutrophil antibody-associated vasculitis is being considered, the clinical symptoms include systemic symptoms (e.g., fever, arthralgia, etc.) and other organ damage. Treatment is usually glucocorticoids (dexamethasone) combined with immunosuppressive drugs (cyclophosphamide). It is important to note that antineutrophil antibody-associated vasculitis is a progressive disease that requires early diagnosis and standardized treatment, especially in children.