Nephrology Section, Department of Medicine, Hasheminejad Kidney Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Iran J Kidney Dis. 2024 Jan;1(1):9-17.
Pauci-immune crescentic glomerulonephritis (GN) is the most common cause of rapidly progressive GN in adults. The aim of this study was to determine the outcome of patients with pauci-immune crescentic GN and risk factors of the development of end-stage kidney disease (ESKD) in these patients.
This case series study was carried on 120 patients with pauci-immune crescentic GN biopsied in our center betwen 1998 and 2016. Inclusion criteria were age > 16 years, at least one crescentic glomerulus, maximally 1+ deposition of immunoglobulins and complement components at fluorescent microscopy, and at least 6 months follow-up. The main outcomes were ESKD and death.
The study population included 120 patients with pauciimmune crescentic GN (mean age was 47 ± 17 years and 49.1% male). There was no significant difference in outcome between patients with diffuse or focal crescentic GN. Seventy-two patients (60%) developed ESKD and 31 patients (25.8%) died. The need for dialysis at admission, lower baseline hemoglobin and GFR and GFR at four months and high percentage of glomerulosclerosis and interstitial fibrosis had a significant relationship with low kidney survival (P < .05). The rate of ESKD was higher in patients who did not receive cyclophosphamide therapy, due to focal crescentic GN or high chronicity, compared to patients who received it (70.7 vs. 28.5%, P < .001).
In our study, a high percentage of patients with pauciimmune crescentic GN developed ESKD. Low first GFR and high chronicity in biopsy were associated with lower kidney survival. Failure to administer cyclophosphamide in seemingly limited or advanced cases, together with late referral may have led to poor prognosis. DOI: 10.52547/ijkd.7545.
寡免疫性新月体肾小球肾炎（GN）是成人急进性 GN 的最常见原因。本研究旨在确定寡免疫性新月体 GN 患者的结局和这些患者发生终末期肾病（ESKD）的危险因素。
本病例系列研究纳入了 1998 年至 2016 年间在我院行肾活检的 120 例寡免疫性新月体 GN 患者。纳入标准为年龄>16 岁，至少一个新月体，荧光显微镜下免疫球蛋白和补体成分最大沉积 1+，且随访时间至少 6 个月。主要结局为 ESKD 和死亡。
研究人群包括 120 例寡免疫性新月体 GN 患者（平均年龄 47±17 岁，49.1%为男性）。弥漫性或局灶性新月体 GN 患者的结局无显著差异。72 例（60%）患者发展为 ESKD，31 例（25.8%）患者死亡。入院时需要透析、基线时血红蛋白和肾小球滤过率（GFR）较低、4 个月时 GFR 较低以及肾小球硬化和间质纤维化比例较高与肾脏存活率较低有显著关系（P<.05）。与接受环磷酰胺治疗的患者相比，未接受环磷酰胺治疗的患者（因局灶性新月体 GN 或高慢性率）ESKD 发生率更高（70.7% vs. 28.5%，P<.001）。
在本研究中，高比例的寡免疫性新月体 GN 患者发展为 ESKD。活检中初次 GFR 较低和慢性率较高与肾脏存活率较低相关。在看似局限或进展期病例中未能应用环磷酰胺治疗，以及延迟转诊，可能导致不良预后。DOI：10.52547/ijkd.7545.
