Pillebout Evangéline, Nochy Dominique
Hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Nephrol Ther. 2010 Nov;6(6):545-57. doi: 10.1016/j.nephro.2010.03.007. Epub 2010 Apr 15.
IgA nephropathy is the primitive glomerulonephritis the most frequently encountered worldwide. In about one case out of three, it is responsible for the progression from progressive renal failure to end-stage renal failure. The pathophysiological mechanisms of this disease which is mediated by immune complexes remain unclear. The presentation, clinical progression and optical microscope aspect of the renal biopsy may widely vary, making any histological classification very difficult. Most therapeutic studies include the patients only on clinical criteria of severity. The only consensual management is that of patients with a nephropathy and mild glomerular lesions and a nephritic syndrome, or with an extracapillar glomerulonephritis and a rapidly progressive renal failure; corticoids are indicated in former cases while corticoids must be combined with immunosuppressive agents in the latter ones. Corticotherapy may be considered in patients with a proteinuria higher than 1g/day without renal failure. In any patient with primitive IgA nephropathy, the overall management used for chronic glomerulopathy must be initiated, including, in case of arterial hypertension or proteinuria, the renin-angiotensin system blockade.
IgA肾病是全球最常见的原发性肾小球肾炎。大约三分之一的病例中，它是导致从进行性肾衰竭发展至终末期肾衰竭的原因。这种由免疫复合物介导的疾病的病理生理机制仍不清楚。肾活检的表现、临床进展和光学显微镜下表现可能差异很大，使得任何组织学分类都非常困难。大多数治疗研究仅根据临床严重程度标准纳入患者。唯一达成共识的治疗方案是针对患有肾病且肾小球病变轻微和肾炎综合征的患者，或患有毛细血管外肾小球肾炎和快速进行性肾衰竭的患者；前一种情况使用皮质类固醇，而后一种情况皮质类固醇必须与免疫抑制剂联合使用。对于蛋白尿高于1g/天且无肾衰竭的患者可考虑使用皮质激素治疗。对于任何原发性IgA肾病患者，必须启动用于慢性肾小球病的整体治疗方案，包括在存在动脉高血压或蛋白尿的情况下进行肾素-血管紧张素系统阻断。
