Revisiting post-infectious glomerulonephritis in the emerging era of C3 glomerulopathy.
Revisiting post-infectious glomerulonephritis in the emerging era of C3 glomerulopathy.
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DOI：
10.1093/ckj/sfw032
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发表时间：
2016-06
影响因子：
4.6
通讯作者：
Chang A
中科院分区：
文献类型：
--
作者：
Khalighi MA;Wang S;Henriksen KJ;Bock M;Keswani M;Meehan SM;Chang A
感染后肾小球肾炎（PIGN）是一种免疫复合物介导的肾小球损伤，通常会消退。显性C3沉积是PIGN的特征，但随着C3肾小球肾炎（C3 GN）作为一个独特的实体的出现，目前还不清楚PIGN和C3 GN之间的病理相似性应如何协调。因此，肾脏病学家和肾脏病理学家在活检时需要额外的指导。我们研究了23例诊断为PIGN的儿童和年轻成人患者。患者被分为两组，一组C3和免疫球蛋白之间的共显性，另一组符合C3 GN的诊断标准。比较两组的临床和病理特点。没有临床和/或病理特征可以区分C3共显性沉积和C3显性沉积。两组中几乎所有患者均在未进行临床干预的情况下恢复了基线肾功能。虽然补体旁路途径异常的识别是C3 GN的特征，但检测并不广泛，周转时间通常超过1个月。我们的研究发现，在一组年轻患者中，PIGN伴C3沉积的患者具有良好的短期结局。建议密切临床观察持续性异常，如低补体血症、长期血尿或蛋白尿，以挑出可能存在内源性补体异常的患者。
Post-infectious glomerulonephritis (PIGN) is an immune complex-mediated glomerular injury that typically resolves. Dominant C3 deposition is characteristic of PIGN, but with the emergence of C3 glomerulonephritis (C3GN) as a distinct entity, it is unclear how the pathologic similarities between PIGN and C3GN should be reconciled. Therefore, nephrologists and nephropathologists need additional guidance at the time of biopsy. We studied 23 pediatric and young adult patients diagnosed with PIGN. Patients were divided into two groups, one with co-dominance between C3 and immunoglobulins and the other meeting proposed diagnostic criteria for C3GN. Clinical and pathological features were compared. No clinical and/or pathological features could distinguish between those with C3-co-dominant deposits and those with C3 dominance. Nearly all patients in both groups regained their baseline renal function without clinical intervention. Although the identification of abnormalities of the alternative pathway of complement is characteristic of C3GN, testing is not widely available and the turnaround time often exceeds 1 month. Our study found that PIGN with either co-dominant or dominant C3 deposition in a cohort of young patients has excellent short-term outcomes. Close clinical observation for persistent abnormalities, such as hypocomplementemia, prolonged hematuria or proteinuria, is recommended to single out patients that may harbor intrinsic complement abnormalities.