Department of Renal Medicine, The Canberra Hospital, Canberra, ACT, Australia.
Department of Immunology and Infectious Disease, John Curtin School of Medical Research, Canberra, ACT, Australia.
BMC Nephrol. 2020 Jul 11;21(1):265. doi: 10.1186/s12882-020-01923-5.
C3 glomerulonephritis is a recently described entity with heterogeneous histopathological features. This study was conducted to assess the effect of reclassification of C3 glomerulopathies on renal outcomes, mortality, and response to therapy.
We undertook a retrospective analysis of 857 renal biopsies collected at The Canberra Hospital. Samples with predominant C3 staining were reviewed by a renal histopathologist. Of 31 biopsies with predominant C3 staining, 10 fulfilled histological criteria for C3 glomerulonephritis, while the remaining 21 cases were used as C3 Controls.
Aside from a higher incidence of C3 glomerulonephritis in Torres Strait islanders (40% vs 5% C3 Controls, p = 0.04), presentation demographics were similar between the two groups. Median creatinine at diagnosis was higher in patients with C3 glomerulonephritis (253 umol/L IQR 103-333 vs 127 umol/L C3 Controls, IQR 105-182, p = 0.01). Prior to reclassification, a majority of C3 glomerulonephritis cases were diagnosed as membranoproliferative glomerulonephritis (60% vs 5% (C3 Controls) p < 0.01). Electron microscopy demonstrated all C3 glomerulonephritis patients had C3 deposition (100% vs 38% p = 0.02), these deposits were amorphous in nature (50% vs 5% respectively p = 0.007). C3 glomerulonephritis patients had shorter median follow-up (405 days IQR 203-1197 vs 1822 days respectively, IQR 1243-3948, p = 0.02). Mortality was higher in C3 glomerulonephritis patients (30% vs 14% in C3 Controls (log rank p = 0.02)).
We have devised a diagnostic and treatment algorithm based on the results of literature review and our current study. Further prospective assessment is required to review diagnostic and treatment outcomes for this disease in Australian centres.
C3 肾小球肾炎是一种新近描述的疾病实体，具有异质性的组织病理学特征。本研究旨在评估 C3 肾小球疾病的重新分类对肾脏结局、死亡率和治疗反应的影响。
我们对堪培拉医院收集的 857 例肾活检进行了回顾性分析。对主要 C3 染色的样本由肾脏组织病理学家进行了回顾。在 31 例主要 C3 染色的活检中，10 例符合 C3 肾小球肾炎的组织学标准，而其余 21 例作为 C3 对照组。
除了托雷斯海峡岛民中 C3 肾小球肾炎的发病率较高（40% vs 5% C3 对照组，p=0.04）外，两组患者的临床表现相似。C3 肾小球肾炎患者的诊断时肌酐中位数更高（253μmol/L IQR 103-333 比 127μmol/L C3 对照组 IQR 105-182，p=0.01）。在重新分类之前，大多数 C3 肾小球肾炎病例被诊断为膜增生性肾小球肾炎（60% vs 5%（C3 对照组），p<0.01）。电子显微镜显示所有 C3 肾小球肾炎患者均有 C3 沉积（100% vs 38%，p=0.02），这些沉积物呈非晶态（50% vs 5%，p=0.007）。C3 肾小球肾炎患者的中位随访时间更短（405 天 IQR 203-1197 与 1822 天 IQR 1243-3948 相比，p=0.02）。C3 肾小球肾炎患者的死亡率更高（30% vs 14% C3 对照组（对数秩检验 p=0.02））。
我们根据文献回顾和本研究的结果制定了诊断和治疗方案。需要进一步的前瞻性评估来审查澳大利亚中心这种疾病的诊断和治疗结果。
