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13 例原发性干燥综合征患者经活检证实的肾脏受累及预后。

Biopsy-proven renal involvement and prognosis in 13 hispanic patients with primary Sjögren syndrome.

机构信息

Departamento de Nefrología y Metabolismo Mineral, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, México.

Departamento de Nefrología, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

出版信息

Med Clin (Barc). 2018 Jan 23;150(2):43-48. doi: 10.1016/j.medcli.2017.06.050. Epub 2017 Aug 30.

Abstract

BACKGROUND

The aim of this study was to describe a case series of 13 Hispanic patients with primary Sjögren syndrome (pSS) and biopsy-proven renal involvement.

METHODS

We describe the clinical, serological and histological characteristics as well as the prognosis in a group of patients with pSS and biopsy-proven renal involvement, treated in 2 referral nephrology units in Mexico City.

RESULTS

Thirteen patients with pSS underwent kidney biopsy (KB) over a period of 27 years. The median duration from pSS diagnosis to KB was 13.9 months. Seven patients (54%) had glomerulonephritis and 6 patients (46%) had tubulointerstitial nephritis. All patients were treated with corticosteroids and/or immunosuppressants. Eight patients (62%) remained stable or their renal function improved after a median follow-up of 12 months.

CONCLUSIONS

This case series reflects the broad spectrum of renal involvement in pSS. We observed that in our Hispanic population, glomerular involvement was the most frequent abnormality, mainly membranous glomerulopathy, followed by tubulointerstitial disease. Tubular atrophy and interstitial fibrosis were also common biopsy findings. Treatment with corticosteroids or other immunosuppressive agents appear to slow renal disease progression.

摘要

背景

本研究旨在描述 13 例经活检证实的原发性干燥综合征(pSS)合并肾脏受累的西班牙裔患者的病例系列。

方法

我们描述了一组在墨西哥城 2 个转诊肾病单位接受治疗的 pSS 合并经活检证实的肾脏受累患者的临床、血清学和组织学特征以及预后。

结果

13 例 pSS 患者在 27 年内接受了肾脏活检(KB)。从 pSS 诊断到 KB 的中位时间为 13.9 个月。7 例患者(54%)有肾小球肾炎,6 例患者(46%)有肾小管间质性肾炎。所有患者均接受了皮质类固醇和/或免疫抑制剂治疗。在中位随访 12 个月后,8 例患者(62%)病情稳定或肾功能改善。

结论

本病例系列反映了 pSS 广泛的肾脏受累谱。我们观察到,在我们的西班牙裔人群中,肾小球受累是最常见的异常,主要是膜性肾小球病,其次是肾小管间质性疾病。肾小管萎缩和间质纤维化也是常见的活检发现。皮质类固醇或其他免疫抑制剂的治疗似乎可以减缓肾脏疾病的进展。

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