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145 例疑似良性免疫球蛋白 A 肾病患者的长期预后。

Long-term outcome in 145 patients with assumed benign immunoglobulin A nephropathy.

机构信息

Renal Research Group, Department of Clinical Medicine, University of Bergen, Norway.

Department of Medicine, Haukeland University Hospital, Bergen, Norway.

出版信息

Nephrol Dial Transplant. 2017 Nov 1;32(11):1841-1850. doi: 10.1093/ndt/gfx242.

DOI:10.1093/ndt/gfx242
PMID:29106593
Abstract

BACKGROUND

Patients with immunoglobulin A nephropathy (IgAN) who present with mild to moderate proteinuria and normal renal function are assumed to have excellent short-term renal prognosis, but the long-term prognosis is uncertain.

METHODS

Patients were selected from the Norwegian Kidney Biopsy Registry based on the following criteria: diagnostic renal biopsy performed in the period 1988-99, with estimated glomerular filtration rate (eGFR) ≥60 mL/min/1.73 m2 and proteinuria <1 g/24 h at the time of biopsy. Patients were invited for a nephrological examination with a review of medical history and investigation of blood pressure, urinary findings and eGFR.

RESULTS

A total of 145 patients attended the examination, performed by the first author, after a median of 22 (interquartile range 19-25) years after diagnosis. At the examination, 27 patients (18.6%) had a ≥50% decrease in GFR, of whom 4 (2.8%) had developed end-stage renal disease (ESRD). The mean duration from renal biopsy to ≥ 50% decrease in GFR was 17.3 ± 5.1 years in our cohort. Clinical remission was observed in 42 (29.0%) patients. Renal biopsies were re-examined utilizing the Oxford classification criteria. Mesangial hypercellularity was found in 12.3%, endocapillary proliferation was detected in 10.7% and segmental glomerulosclerosis was observed in 23.8%. All biopsies were scored as T0 (tubular atrophy in < 25% of the cortical area). None of the clinical or histopathological variables recorded at the time of biopsy could identify patients with progressive disease. Cumulative risks of ≥50% decrease in eGFR were 2.1% after 10 years, 4.1% after 15 years, 13.9% after 20 years and 24.7% after 25 years.

CONCLUSIONS

We have shown that 18.6% of patients with assumed benign IgAN had progressive disease after a median duration of 22 years and that these patients could not be predicted at the time of biopsy. Our study demonstrates that an extended follow-up period is needed when assessing prognosis in this group of patients.

摘要

背景

患有免疫球蛋白 A 肾病(IgAN)且表现为轻度至中度蛋白尿和正常肾功能的患者被认为具有极好的短期肾脏预后,但长期预后不确定。

方法

根据以下标准从挪威肾脏活检登记处选择患者:1988-99 年期间进行诊断性肾脏活检,活检时估计肾小球滤过率(eGFR)≥60mL/min/1.73m2,蛋白尿<1g/24h。患者被邀请进行肾脏检查,回顾病史并检查血压、尿液发现和 eGFR。

结果

中位随访 22 年后(19-25 年),共 145 例患者接受了由第一作者进行的检查。在检查时,27 例(18.6%)患者的肾小球滤过率下降了≥50%,其中 4 例(2.8%)已发展为终末期肾病(ESRD)。从肾脏活检到肾小球滤过率下降≥50%的平均时间为 17.3±5.1 年。我们的队列中,42 例(29.0%)患者达到临床缓解。利用牛津分类标准重新检查肾脏活检。发现系膜细胞增生 12.3%,内皮下增殖 10.7%,节段性肾小球硬化 23.8%。所有活检均评分为 T0(<25%皮质区的肾小管萎缩)。在活检时记录的任何临床或组织病理学变量都无法识别进展性疾病患者。eGFR 下降≥50%的累积风险为 10 年后 2.1%,15 年后 4.1%,20 年后 13.9%,25 年后 24.7%。

结论

我们表明,在中位随访 22 年后,认为良性 IgAN 的 18.6%患者发生了进行性疾病,而这些患者在活检时无法预测。我们的研究表明,在评估这组患者的预后时需要延长随访时间。

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