关键词: 小血管炎, 抗中性粒细胞胞浆抗体, 免疫抑制剂, 糖皮质激素

Abstract:

Primary small vessel vasculitis has been pathologically classified as a spectrum of autoimmune diseases involving the kidneys and lungs.Treatment of primary small vessel vasculitis，based on the KDIGO guideline，included initial and maintenance therapy.Initial treatment consisted mainly of corticosteroids and cytotoxic medications (esp.cyclophosphamide，rituximab) and may involve highdose methylprednisolone and plasma exchange therapy as an emergent surrogate in severe cases，while the maintenance therapy mostly included the immunosuppressants，typically，azathioprine and mycophenolate mofetil.The treatment could be initiated by increasing the doses of corticosteroids or immunosuppressants according to the severity of relapse and，if warranted，repeat the induction protocol for initial therapy.

Key words: vasculitis, anti-neutrophil cytoplasmic antibody, immunosuppressant, glucocorticoid