Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-Cho, Kashihara, Nara, Japan.
Department of Diagnostic Pathology, Kobe City Medical Center General Hospital, Kobe, Japan.
Virchows Arch. 2020 Oct;477(4):565-572. doi: 10.1007/s00428-020-02800-0. Epub 2020 Apr 16.
IgA nephropathy (IgAN) is common chronic glomerulonephritis with variable prognosis, ranging from minor urinary abnormalities to end-stage renal disease. The revised Oxford classification of IgAN explains that cellular/fibrocellular crescents are associated with poor renal prognosis, proposing an extension to the MEST-C score. C3 immunofluorescent staining follows a distribution similar to IgA staining. Therefore, complement activation was reported to play a pivotal role in IgAN pathogenesis. This study included 132 IgAN patients diagnosed by renal biopsies. The clinical parameters at the time of the biopsies were obtained from patient data records. We classified the patients into C ≥ 1 and C0 groups, and compared clinical, light microscopic, and immunofluorescent features. In the C ≥ 1 group, 2 (1.5%) and 31 (23.5%) patients were assigned to C2 and C1, respectively. The remaining 99 patients (75%) were classified as C0. The C ≥ 1 group had lower average age and rate of hypertension, and higher score of urinary occult blood and E score. The C ≥ 1 group had significantly higher average immunofluorescence scores for IgA, C5b-9, mannose-associated serine protease (MASP) 1/3, MASP2, properdin, factor B, and kappa. The steroid use rate was significantly higher in the C ≥ 1 group. During the follow-up period of 2.90 years on average, the rate of renal dysfunction was not significantly different between groups. Crescent formation in IgAN was associated with activation of the lectin and alternative pathways. The C ≥ 1 group had significantly increased use of steroids, which probably caused comparable renal function during the follow-up period.
IgA 肾病(IgAN)是一种常见的慢性肾小球肾炎,其预后各不相同,从轻度尿异常到终末期肾病不等。IgAN 的修订版牛津分类法解释说,细胞/成纤维细胞新月体与不良的肾脏预后相关,建议扩展 MEST-C 评分。C3 免疫荧光染色的分布与 IgA 染色相似。因此,补体激活被报道在 IgAN 发病机制中起关键作用。本研究纳入了 132 例经肾活检诊断为 IgAN 的患者。活检时的临床参数从患者数据记录中获得。我们将患者分为 C≥1 组和 C0 组,并比较了临床、光镜和免疫荧光特征。在 C≥1 组中,有 2 例(1.5%)和 31 例(23.5%)患者分别被归类为 C2 和 C1。其余 99 例(75%)患者被归类为 C0。C≥1 组的平均年龄和高血压发生率较低,尿潜血和 E 评分较高。C≥1 组的 IgA、C5b-9、甘露糖结合丝氨酸蛋白酶(MASP)1/3、MASP2、备解素、因子 B 和κ的平均免疫荧光评分显著较高。C≥1 组的类固醇使用率显著较高。平均随访 2.90 年后,两组的肾功能障碍发生率无显著差异。IgAN 中的新月体形成与凝集素和替代途径的激活有关。C≥1 组类固醇的使用率显著增加,这可能导致在随访期间具有可比的肾功能。