新西兰的 C3 肾小球肾炎——病例系列研究。
C3-glomerulonephritis in New Zealand - a case series.
机构信息
Auckland City Hospital, Auckland, New Zealand.
Department of Renal Medicine, Middlemore Hospital, 100 Hospital Rd, Otahuhu, Auckland, 2025, New Zealand.
出版信息
BMC Nephrol. 2020 Sep 17;21(1):399. doi: 10.1186/s12882-020-02056-5.
BACKGROUND
C3-glomerulonephritis can lead to progressive renal impairment from complement-mediated glomerular injury. Incidence and outcomes of C3-glomerulonephritis are not known in the New Zealand population.
METHODS
We reviewed all cases of C3-glomerulonephritis from the past 10 years at a tertiary referral centre in New Zealand. Descriptive information on baseline characteristics and clinical outcomes was collected.
RESULTS
Twenty-six patients were included (16 men; mean ± SD age 44 ± 25 years) with a median follow-up of 30 months. Disease incidence was 1.3 cases per million individuals, of which 42% were Pacific Islanders. Most patients presented with renal impairment, with a median (IQR) creatinine at diagnosis of 210 (146-300) μmol/L, and 11 (42%) patients presented with nephrotic syndrome. Seven (27%) patients progressed to end stage renal disease and 2 (8%) had died. End stage renal disease occurred in 20% of patients treated with immunosuppression and in 50% of those not treated. Complete remission was seen in 25% of patients treated with some form of immunosuppression and in 17% of those not treated.
CONCLUSIONS
Our results are consistent with previous descriptions of C3-glomerulonephritis. There was a suggestion of better clinical outcomes in patients treated with immunosuppression. There was a higher disease incidence in Pacific Islanders, which may indicate an underlying susceptibility to complement dysfunction in this population.
背景
C3 肾小球肾炎可导致补体介导的肾小球损伤引起进行性肾损伤。在新西兰人群中，C3 肾小球肾炎的发病率和结局尚不清楚。
方法
我们回顾了新西兰一家三级转诊中心过去 10 年中所有的 C3 肾小球肾炎病例。收集了基线特征和临床结局的描述性信息。
结果
共纳入 26 例患者（男性 16 例；平均年龄 44 ± 25 岁），中位随访 30 个月。疾病发病率为每百万个体 1.3 例，其中 42%为太平洋岛民。大多数患者表现为肾功能损害，诊断时肌酐中位数（IQR）为 210（146-300）μmol/L，11 例（42%）患者表现为肾病综合征。7 例（27%）患者进展为终末期肾病，2 例（8%）患者死亡。接受免疫抑制治疗的患者中有 20%进展为终末期肾病，而未接受治疗的患者中有 50%进展为终末期肾病。接受某种形式免疫抑制治疗的患者中有 25%达到完全缓解，而未接受治疗的患者中有 17%达到完全缓解。
结论
我们的结果与之前对 C3 肾小球肾炎的描述一致。接受免疫抑制治疗的患者临床结局更好。太平洋岛民的疾病发病率较高，这可能表明该人群补体功能障碍的潜在易感性。
Zotero 插件