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目的：显微镜下多血管炎（microscopic polyangiitis，MPA）通常易侵犯肾脏，很少累及神经系统，而同时累及肺及中枢神经系统的重症病例较少见。本文旨在探讨重症显微镜下多血管炎（MPA)的临床表现、诊断与治疗，为临床提供借鉴。方法：回顾性分析重症显微镜下多血管炎（MPA)的文献资料，结合本中心病例，54 岁女性，尿检异常6 年，咳血3 年因肾功能衰竭合并心衰入院。结合实验室检查及影像学改变，诊断为重症AASV，考虑MPA可能性大。结果：入院后即给予激素联合环磷酰胺冲击治疗，病情明显好转；3周后出现病情反复，再次激素冲击治疗，效果不如前。继而出现脑出血，病情进一步恶化，最终因肾、肺、脑等多器官功能衰竭于入院5 周后死亡。结论：对MPA 患者关键在于早发现早治疗；而对缓解重症MPA 患者病情，激素联合环磷酰胺冲击治疗在初始阶段有效。

英文摘要:

Objective:Microscopic polyangiitis (MPA) rarely affects the central nervous system. Renal disease is almost universally present but serious cases involving lung and central nervous system are not commonly reported. This paper aims to discuss clinical diagnosis and treatment of serious MPA, provided the clinical reference.Methods:Retrospective analysis under serious microscopic polyangiitis (MPA) reference data, we reported a 54-year-old female, presented with a 6-year history of abnormal urinalysis and 3-year hemoptysis, hospitalized on account of renal failure complicated by heart failure. A severe AASV (most possibly MPA) diagnosis was made by laboratory tests and imaging changes. Results:Following the intravenous pulse administration of Methylprednisolone (MP) and cyclophosphamide (CTX), both symptoms and imaging examination were improved obviously soon after admission. However, MP pulse therapy could not prevent the disease from aggravating again from the third week after admission.The disease got worse as cerebral hemorrhage, then the patient died of multiple organs（kidney、lung and brain）failure after five weeks hospitalization at last.Conclusion:It is important for patients with MPA to diagnosis and treatment as earlier as possible, MP and CTX pulse therapy is effective in improving the symptoms of the serious MPA in the early stage.

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