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免疫球蛋白 A 肾病伴新月体在多民族东南亚队列中的临床病程。

Clinical course of Immunoglobulin A nephropathy with crescents in a multi-ethnic Southeast Asian cohort.

机构信息

Department of Renal Medicine, Singapore General Hospital, Singapore, Singapore.

Department of General Medicine, Sengkang General Hospital, Singapore, Singapore.

出版信息

Nephrology (Carlton). 2020 Sep;25(9):708-713. doi: 10.1111/nep.13723. Epub 2020 Jun 8.

Abstract

AIM

Clinical presentation and course of Immunoglobulin A nephropathy vary by ethnicity and geography and significance of extracapillary proliferation or crescents (IgAN-C) in Southeast Asia is not well described. We aimed to describe the clinical course of IgAN-C in Singapore.

METHODS

Retrospective cohort study of adult biopsy-proven IgAN diagnosed between February 2011 and October 2016 in 2 hospital-based nephrology units. Outcome was chronic kidney disease (CKD) progression, defined as reduction in eGFR ≥50% or end stage renal failure (ESRF).

RESULTS

One hundred and forty-five patients were studied. Among individuals with IgAN-C (n = 44, 30%), 38 patients had cellular or fibrocellular crescents in 1 to 25% of the glomeruli and 6 had crescents in >25%. Median eGFR was 54 (33, 83) mL/min/1.73 m . Compared to IgAN without crescents, IgAN-C had greater proteinuria (median 2.9 [1.4, 5.4] g/g vs 1.9 [1.1, 3.6] g/g, P = .03) and more had endocapillary hypercellularity (96% vs 39%, P < .001). IgAN-C were also more likely to receive immunosuppressants (66% vs 43%, P = .01) such as prednisolone (63% vs 38%, P = .006) and cyclophosphamide (12% vs 2%, P = .03). Median follow up was 27 (12, 46) months. IgAN-C were more likely to achieve proteinuria reduction ≥50% at 6 months (66% vs 44%, P = .03). CKD progression within 12 months was not different among those with and without crescents (13% vs 10% respectively, P = .73). However, immunosuppressant treatment of IgAN-C was associated with reduced ESRF (0 vs 20%, P = .03).

CONCLUSION

Immunosuppressants may attenuate the risk of ESRF in IgAN-C.

摘要

目的

免疫球蛋白 A 肾病(IgAN)的临床表现和病程因种族和地理位置而异,而东亚毛细血管外增殖或新月体(IgAN-C)的意义尚未得到充分描述。本研究旨在描述新加坡 IgAN-C 的临床病程。

方法

对 2011 年 2 月至 2016 年 10 月期间在 2 家医院肾脏病科确诊的成人活检证实为 IgAN 的患者进行回顾性队列研究。结局为慢性肾脏病(CKD)进展,定义为 eGFR 下降≥50%或终末期肾病(ESRD)。

结果

共纳入 145 例患者。在 IgAN-C 患者(n=44,30%)中,38 例患者肾小球 1%至 25%范围内存在细胞性或纤维细胞性新月体,6 例患者肾小球 25%以上存在新月体。中位 eGFR 为 54(33,83)mL/min/1.73m2。与无新月体的 IgAN 相比,IgAN-C 的蛋白尿更多(中位数 2.9[1.4,5.4]g/g 比 1.9[1.1,3.6]g/g,P=0.03),内皮下细胞增多更为常见(96%比 39%,P<0.001)。IgAN-C 更可能接受免疫抑制剂治疗(66%比 43%,P=0.01),如泼尼松龙(63%比 38%,P=0.006)和环磷酰胺(12%比 2%,P=0.03)。中位随访时间为 27(12,46)个月。IgAN-C 在 6 个月时蛋白尿减少≥50%的可能性更大(66%比 44%,P=0.03)。在有新月体和无新月体的患者中,12 个月内 CKD 进展的比例没有差异(分别为 13%和 10%,P=0.73)。然而,IgAN-C 的免疫抑制剂治疗与 ESRF 降低相关(0 比 20%,P=0.03)。

结论

免疫抑制剂可能降低 IgAN-C 发生 ESRF 的风险。

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