肉芽肿性多血管炎的诊治

Diagnosis and treatment of granulomatosis with polyangiitis

肉芽肿性多血管炎（GPA）是一种多器官受累的坏死性肉芽肿性血管炎，主要侵犯上、下呼吸道和肾脏。GPA是一种少见病，临床表现复杂多样，诊断困难，临床上容易误诊。重症患者病死率高，目前多采用糖皮质激素联合免疫抑制剂治疗，分诱导缓解治疗和维持缓解治疗两个阶段。现对GPA的临床表现、诊断方法和治疗方案进行综述，以提高临床医生对GPA的进一步认识，减少误诊和误治。

Granulomatosis with polyangiitis （GPA） is a systemic inflammatory disease characterized by necrotizing granulomatous inflammation and small vessel vasculitis. Upper and/or lower respiratory tract and kidneys are involved mainly. GPA is a rare disease with complicated clinical manifestations, which causes difficulty in making correct diagnosis. GPA is a serious disease, with a nearly always fatal outcome in the absence of treatment. The combination of immunosuppressant drugs and corticosteroids is a standardized treatment for GPA. Treatment is based on two phases known as the induction phase and the maintenance phase. Here, the diagnosis and treatment of GPA are reviewed.