Bindi P, Gilson B, Aymard B, Brillet G, Birembaut P, Joly D
Service de Néphrologie, Médecine interne, Centre Hospitalier, Verdun.
Nephrologie. 1993;14(2):95-100.
Forty-two cases of pauci-immune necrotizing glomerulonephritis were reviewed on a 10 years period. Selection was exclusively based on histological criteria, i.e. at least one elementary lesion of extracapillary proliferation and/or glomerular necrosis, without immunoglobulin deposits. Mean age was 56. Thirty per cent of patients presented with normal or non-worsening renal function. At least one extra-renal sign was present in 66% of patients. ANCA were found in 9/20 cases. Death occurred in 12 patients. Age over 60 and oligo anuria were the most predictive factors for the fatal outcome. Half of the patients were on dialysis at the end of their follow-up. The renal function at presentation was the main predictive variable for renal outcome. Severe tubular necrosis was associated with a poorer renal evolution whereas treatment with cyclophosphamide led to frequent improvement in this retrospective study.
在10年期间对42例寡免疫性坏死性肾小球肾炎病例进行了回顾性研究。病例选择完全基于组织学标准，即至少有一项毛细血管外增生和/或肾小球坏死的基本病变，且无免疫球蛋白沉积。平均年龄为56岁。30%的患者肾功能正常或无恶化。66%的患者至少有一项肾外表现。20例中有9例检测到抗中性粒细胞胞浆抗体（ANCA）。12例患者死亡。年龄超过60岁和少尿无尿是死亡结局的最主要预测因素。随访结束时，一半的患者接受透析治疗。就诊时的肾功能是肾脏预后的主要预测变量。严重肾小管坏死与较差的肾脏转归相关，而在这项回顾性研究中，环磷酰胺治疗常使病情改善。
