ANCA-associated vasculitis is a group of diseases marked by inflammation and necrosis of small and medium-sized vessels, mainly including Wegener’s granulomatosis, Churg Strauss syndrome, and microscopic polyangiitis.In 2007 the British Society for Rheumatology (BSR) published a guideline for the treatment of ANCA-associated vasculitis. Currently, vasculitis treatment includes treatment during the induction of remission and maintenance of remission periods, with the main regimen based on disease severity and extent. The guideline classifies vasculitis into 3 groups of types, namely: (1) local and/or early type; (2) systemic type with organ damage; and (3) severe type with life-threatening disease. 1. Local and/or early type: The first line of treatment is cyclophosphamide or methotrexate, of which methotrexate has a higher recurrence rate, and cyclophosphamide is used for disease progression, recurrence or local destruction. 2. Systemic type with organ damage: The first-line therapeutic agents are cyclophosphamide and glucocorticoids. The former often results in higher total cyclophosphamide and a significantly higher risk of infection, but there is no significant difference in clinical remission rates and recurrence rates between these two regimens. If clinical remission is achieved with 3 months of low-dose oral cyclophosphamide and 3 to 6 months of intravenous cyclophosphamide shocks, a switch to maintenance therapy should be made. The maximum treatment period is 6 months if clinical remission is achieved regardless of which induction regimen is taken. 3. Severe type: If combined with severe renal impairment (blood creatinine >500umol/L), cyclophosphamide (oral low dose or intravenous shock) and glucocorticoids should be given in combination with plasma exchange. In life-threatening situations (e.g., pulmonary hemorrhage), plasma exchange should also be administered. The treatment guidelines also state that glucocorticosteroids should generally be administered orally daily at a starting dose of 1 mg/Kg/d and a maximum dose of 60 mg, with methylprednisolone shocks (250-500 mg) if necessary. If cyclophosphamide is not tolerated, methotrexate, azathioprine, leflunomide or mycophenolate may be considered. Prednisone volume and immunosuppressive dose may be increased in mild relapses, while cyclophosphamide and increased prednisone volume should be given in severe relapses; methylprednisolone shock or plasma exchange may also be considered. The efficacy of infliximab, intravenous immunoglobulin, anti-thymocyte globulin, anti-CD52 monoclonal antibody, deoxynivalenol and melphalan in patients with refractory vasculitis is still under investigation, but possible causes of persistent disease, disease relapse or malignancy and infection should be identified. Disease relapse can occur at any time after clinical diagnosis and induction of remission and requires assessment of disease activity and extent of lesions. Because ANCA titers do not necessarily correlate with disease activity, patients with only elevated ANCA should not be intensified. In contrast, patients with persistently positive ANCA may cause relapse if the drug is discontinued. This treatment guideline suggests that those using immunosuppressive therapy should take the following 10 measures: (1) Mestinon sodium prevents adverse events of urinary tract epithelial toxicity; (2) methotrexate/sulfamethoxazole prevents Pneumocystis carinii infection; (3) antifungal agents should be applied prophylactically; (3) mupirocin should be used for prolonged nasal Staphylococcus aureus infection; (4) female patients should be tested regularly for cervical epithelium to prevent cervical intraepithelial neoplasia; (5) the possibility of infertility should be considered in those applying cyclophosphamide; (6) all patients treated with high doses of glucocorticoids should be prevented from osteoporosis; (7) all patients treated with immunosuppressive therapy should be tested regularly for tuberculosis; (9) all patients treated with immunosuppressive therapy should be vaccinated against pneumococcal infections and influenza; (10) regular evaluation should be made for risk of cardiovascular disease and thrombosis.