ObjectiveTo analyze the clinical,pathological features and long-term follow-up results of patients with ANCA associated glomerulonephritis.Methods40 patients with diagnosed with ANCA associated nephritis from 2006 to 2010 in Ruijin hospital affiliated to Shanghai jiaotong university school of medicine,were retrospectively analyzed the clinical features,pathologic manifestation,auxiliary examination results and the treatment effect,and the prognosis of patients with follow-up of understanding.Results40 cases of patients with ANCA associated glomerulonephritis,the onset of an average age of 54.8±16.6 years,32.5% of them were over 65 years old,underwent renal biopsy,according to pathological type,focal type in 11 cases,hardening 10 cases,12 cases of mixed type,crescent shape 7 cases,37 cases be hormones and immunosuppressive therapy,3 cases of hormone therapy alone,induction of remission of 30 cases were treated with steroid pulse therapy combined with cyclophosphamide,7 cases be combined hormone mycophenolate mofetil therapy,renal function combined with a sharp progressors after double membrane plasmapheresis and hemodialysis,severe to be gamma globulin treatment,six months,8 cases of complete remission,partial remission in 26 cases,6 cases no remission,overall response rate 85%. Follow-up observation 78.2±17.2 months,4 patients died,4 cases were lost,32 survived,including 8 cases maintain dialysis. Focal type of kidney damage is relatively light,it showed little chance of acute renal failure. Hardening likely manifestations of chronic renal failure,namely the need to maintain progress to ESRD dialysis treatment three higher proportion than the rest. ConclusionThe clinical manifestations and prognosis of different types of renal pathology and prognosis were significantly different,and ANCA detection and renal biopsy were performed in time. According to the best treatment plan for patients,and actively prevent complications,regular follow-up can greatly improve the survival rate,long-term survival rate and quality of life of patients.%目的：分析ANCA相关性肾炎患者的临床、病理特点和长期随访结果。方法回顾性分析40例ANCA相关性肾炎患者临床特点、病理表现、相关辅助检查结果及治疗效果，并随访了解患者预后情况。结果40例确诊为ANCA相关性肾炎患者，起病时平均年龄（54.8±16.6）岁，其中32.5%的患者≥65岁，均行肾穿刺活检，根据肾脏病理分型，局灶型11例，硬化型10例，混合型12例，新月体型7例，37例予以激素及免疫抑制剂治疗，3例单用激素治疗，诱导缓解期30例患者给予激素联合环磷酰胺冲击治疗，7例予以激素联合吗替麦考酚酯治疗，肾功能急剧进展者辅以双膜血浆置换及血液透析，重症者予以丙种球蛋白治疗，治疗半年后，完全缓解8例，部分缓解26例，未缓解6例，总缓解率85%。随访观察（78.2±17.2）个月，死亡4例，失访4例，存活32例，其中8例维持透析治疗。局灶型肾脏损害相对较轻，表现为急性肾功能衰竭几率小。硬化型表现为慢性肾功能衰竭的可能性较大，即进展为终末期肾病（ESRD）需维持透析治疗的比例较其余三者增高。结论不同肾脏病理类型的临床表现及预后差异较大，及时行ANCA检测及肾穿刺活检，根据患者综合情况实施最佳治疗方案，积极防治并发症，规律随访，可明显提高患者的肾存活率、远期生存率和生活质量。
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机译：Quels sont les problemes ethiques souleves par l'augmentation des capacites cognitives dans le cadre de la defense：cadre legal francais，position ethique du service de sante des armees francais。
