Background

Data on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in children are limited.

Methods

In this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged <18-years with C3 glomerulopathy and IC-MPGN. Initial immunosuppression comprised prednisolone, mycophenolate mofetil (n = 51), tacrolimus (n = 11), and/or IV cyclophosphamide (n = 20). Clinicopathological features, response to therapy, and adverse outcome (eGFR_cr < 15 mL/min/1.73 m² or death) were evaluated.

Results

A total of 92 patients were classified as DDD (n = 48, 52.2%), C3GN (n = 26, 28.3%), and IC-MPGN (n = 18, 19.6%) by immunohistochemistry and electron microscopy; 8 patients with DDD were misclassified as IC-MPGN on immunofluorescence. At last follow-up (median 4.3 years), complete or partial remission occurred in 28.5, 36.1, and 16.7% patients with DDD, C3GN, and IC-MPGN, respectively. Serum albumin at onset < 2.5 g/dL (HR = 0.29, P = 0.005) and persistently low serum C3 (HR = 0.34, P = 0.02) were associated with lack of remission. The 5-year kidney survival was 62.6, 85.5, and 88.5% in patients with DDD, C3GN, and IC-MPGN, respectively (log-rank, P = 0.006). Presentation as rapidly progressive GN (HR = 11.2, P < 0.001), age > 10 years at onset (HR = 4.0, P = 0.004), and DDD (HR = 4.2, P = 0.02) were independently associated with adverse outcome; achieving remission was protective (HR = 0.04; P < 0.001).

Conclusion

Outcome in patients with C3 glomerulopathy and IC-MPGN was unsatisfactory, and only a small proportion of patients achieved complete or partial remission. Patients with DDD were more likely to present with rapidly progressive GN and were at higher risk of adverse outcomes, including kidney failure.

中文翻译：

---

C3肾小球病和免疫复合物膜增生性肾小球肾炎的治疗和结果。

背景

儿童致密沉积病 (DDD)、C3 肾小球肾炎 (C3GN) 和免疫复合物 MPGN (IC-MPGN) 的治疗和结果数据有限。

方法

在这项 2007 年至 2019 年的回顾性单中心研究中，对肾活检进行了审查，纳入了年龄 <18 岁且患有 C3 肾小球病和 IC-MPGN 的患者。初始免疫抑制包括泼尼松龙、吗替麦考酚酯 ( n  = 51)、他克莫司 ( n  = 11) 和/或 IV 环磷酰胺 ( n  = 20)。评估临床病理学特征、对治疗的反应和不良结果（eGFR _cr  < 15 mL/min/1.73 m ²或死亡）。

结果

 免疫组化和电镜检查共92例患者分为DDD（n  = 48, 52.2%）、C3GN（n  = 26, 28.3%）和IC-MPGN（n = 18, 19.6%）；8 名 DDD 患者在免疫荧光上被错误分类为 IC-MPGN。在最后一次随访中（中位 4.3 年），DDD、C3GN 和 IC-MPGN 患者的完全或部分缓解率分别为 28.5%、36.1% 和 16.7%。发病时血清白蛋白 < 2.5 g/dL (HR = 0.29, P  = 0.005) 和持续低血清 C3 (HR = 0.34, P  = 0.02) 与缺乏缓解有关。DDD、C3GN 和 IC-MPGN 患者的 5 年肾脏存活率分别为 62.6%、85.5% 和 88.5%（对数秩，P = 0.006)。表现为快速进展的 GN (HR = 11.2, P  < 0.001)、发病年龄 > 10 岁 (HR = 4.0, P  = 0.004) 和 DDD (HR = 4.2, P  = 0.02) 与不良结局独立相关；达到缓解是保护性的（HR = 0.04；P  < 0.001）。

结论

C3 肾小球病和 IC-MPGN 患者的结果不令人满意，只有一小部分患者达到完全或部分缓解。DDD 患者更有可能出现快速进展的 GN，并且出现包括肾衰竭在内的不良后果的风险更高。