原发性IgA肾病中的微血管损害

Vasculitic lesion in idiopathic IgA nephropathy

目的了解原发性IgA肾病中微血管损害及新月体形成(V/C)的临床、病理特点。方法以2004年确诊并行肾穿刺活检证实的87例伴V/C损害的原发性IgA肾病与同期135例不伴V/C损害的原发性IgA肾病以及伴有V/C的狼疮肾炎患者的临床、病理资料进行比较。结果原发性IgA肾病中较常发生V/C损害,发生率为39.19%;而V/C损害受累小球数占肾小球总数的(14.08±12.75)%。37.9%伴V/C损害的IgA肾病患者血清肌酐升高。血压、尿蛋白等临床指标在有与无V/C损害的两组IgA肾病间均无显著性差异。原发性IgA肾病患者的球性硬化发生率(135例/ 222例,64.86%)、球性硬化数与肾小球总数的比率[(26.98±24.68)%]均显著高于LN组[30例/73 例,40.00%,(16.18±18.80)%]。结论原发性IgA肾病中V/C损害发生率较高,出现常缺乏明显临床表现,并可能导致肾单位的缓慢、持续性、“非显性”丢失,最终进展至终末期肾衰。

Objective To analyze the pathological and clinical characteristics of patients with idiopathic IgA nephropathy accompanied by vasculitic/crescentic lesion (IgA-V/C). Methods Data of 222 patients diagnosed as idiopathic IgA nephropathy by renal biopsy, among them 87 cases with vasculitic/crescenlic (V/C)lesion, from our department in 2004 were analyzed retrospectively. Clinical and pathological data from patients with IgA-V/C were compared to those of non-IgA-V/C patients and of lupus nephritis (LN) patients with V/C lesion. Results Vasculitic/crescentic lesion was found in 39.19% (87/222) patients with idiopathic IgA nephropathy.And about( 14.08±12.75)% of the glomeruli was affected. It should be taken into account that there was no significant differences of clinical manifestations including blood pressure, urinary protein excretion between IgA-V/C group and non-IgA-V/C group .except serum creatinine(Scr)level which was significantly higher in IgA-V/C group. In addition, only 37.9% of IgA-V/C patients presented high Scr level,thus the lesion of V/C in idiopathic IgA nephropathy was easily overlooked. Patients with idiopathic IgA nephropathy were found to have higher percentage of glomerular sclerosis (64.86% vs 40.00%) and ratio of sclerostic glomeruli to total glomeruli [ (26.98 ±24.68)% vs (16.10 ±18.80)% ]as compared to LN group, which further predicated the progressing characteristics of IgA nephropathy. Conclusions Vasculitic/crescentic lesion is a quite common finding in idiopathic IgA nephropathy and often associated with no dramatically symptoms. It is possible for vasculitic/crescentic lesion to induce unmarked lose of nephron slowly and continually, so as to accelerate IgA nephropathy progression to end-stage renal failure.