Complement activation is associated with crescent formation in IgA nephropathy
Complement activation is associated with crescent formation in IgA nephropathy
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DOI：
10.1007/s00428-020-02800-0
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发表时间：
2020-04-16
期刊：
影响因子：
3.5
通讯作者：
Ohbayashi, Chiho
中科院分区：
文献类型：
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作者：
Itami, Hiroe;Hara, Shigeo;Ohbayashi, Chiho
IgA 肾病 (IgAN) 是常见的慢性肾小球肾炎，预后可变，从轻微的尿路异常到终末期肾病。修订后的 IgAN 牛津分类解释了细胞/纤维细胞新月体与肾脏预后不良相关，建议对 MEST-C 评分进行扩展。 C3 免疫荧光染色遵循与 IgA 染色类似的分布。因此，据报道，补体激活在 IgAN 发病机制中发挥关键作用。这项研究包括 132 名通过肾活检诊断的 IgAN 患者。活检时的临床参数是从患者数据记录中获得的。我们将患者分为 C >= 1 和 C0 组，并比较临床、光镜和免疫荧光特征。在 C >= 1 组中，分别有 2 名 (1.5%) 和 31 名 (23.5%) 患者被分配到 C2 和 C1。其余 99 名患者 (75%) 被分类为 C0。 C≥1组平均年龄和高血压发生率较低，尿潜血评分和E评分较高。 C >= 1 组的 IgA、C5b-9、甘露糖相关丝氨酸蛋白酶 (MASP) 1/3、MASP2、备解素、B 因子和 kappa 的平均免疫荧光评分显着较高。 C >= 1 组的类固醇使用率显着较高。在平均2.90年的随访期间，各组之间的肾功能不全发生率没有显着差异。 IgAN 中新月体的形成与凝集素和替代途径的激活有关。 C >= 1 组类固醇的使用显着增加，这可能导致随访期间的肾功能相当。
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.