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73例原发性IgA肾小球肾炎患者重复肾活检的病理病变比较:定量评分的价值及最终预后评估方法

Comparison of pathological lesions on repeated renal biopsies in 73 patients with primary IgA glomerulonephritis: value of quantitative scoring and approach to final prognosis.

作者信息

Alamartine E, Sabatier J C, Berthoux F C

机构信息

Service de Néphrologie, Dialyse et Transplantation Rénale, CHU de Saint-Etienne, Hôpital Nord, Saint-Priest-en-Jarez, France.

出版信息

Clin Nephrol. 1990 Aug;34(2):45-51.

PMID:2225552
Abstract

In order to improve our possibility of establishing a long-term prognosis in IgA nephritis, 73 patients out of a cohort of 282, followed over a mean period of 12 years at the same institution for an IgA nephritis, had a prospective second renal biopsy 5 years later. For all biopsies (RB1 and RB2), we developed a quantitative scoring for all elementary lesions with a glomerular, an interstitial, a tubular and a vascular index. The sum of these 4 indexes gave a global optical score (GOS). Pathological improvement on light microscopy (delta GOS less than or equal to -2) was noticed only in 3 patients (4%), stability (-2 less than delta GOS less than +2) in 30 patients (41%), mild deterioration (+2 less than or equal to GOS less than 5) in 23 patients (32%) and major progression (delta GOS greater than or equal to 5) in 17 patients (23%). We observed no pathological remission, even in the 14 patients with complete clinical remission. The pathological progression was characterized by an increase in all elementary lesions, mainly the tubulo-interstitial and vascular ones. By immunofluorescence mesangial IgA deposits remained stable with no disappearance; however, the number and intensity of vascular C3 deposits were significantly greater on RB2. Chronic renal failure (serum creatinine greater than 1.5 mg/dl) correlated best with major pathological progression and mainly with the progression of extraglomerular lesions. IgA nephritis is a slowly progressive disease with no pathological remission, and its evolution is characterized by progression of extraglomerular lesions, mainly vascular, which might play a major role in the ultimate development of chronic renal failure.

摘要

为了提高我们建立IgA肾病长期预后的可能性,在同一机构对282例IgA肾病患者进行了平均12年的随访,其中73例患者在5年后进行了前瞻性第二次肾活检。对于所有活检(RB1和RB2),我们针对所有基本病变制定了肾小球、间质、肾小管和血管指数的定量评分。这4个指数的总和得出一个整体光学评分(GOS)。仅3例患者(4%)在光学显微镜下出现病理改善(ΔGOS≤ -2),30例患者(41%)病情稳定(-2 < ΔGOS < +2),23例患者(32%)出现轻度恶化(+2 ≤ GOS < 5),17例患者(23%)出现严重进展(ΔGOS≥ 5)。即使在14例临床完全缓解的患者中,我们也未观察到病理缓解。病理进展的特征是所有基本病变增加,主要是肾小管间质和血管病变。通过免疫荧光检查,系膜IgA沉积保持稳定,没有消失;然而,RB2上血管C3沉积的数量和强度明显更大。慢性肾衰竭(血清肌酐>1.5 mg/dl)与严重病理进展的相关性最佳,主要与肾小球外病变的进展相关。IgA肾病是一种缓慢进展的疾病,没有病理缓解,其演变特征是肾小球外病变进展,主要是血管病变,这可能在慢性肾衰竭的最终发展中起主要作用。

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