Primary Sjogren's syndrome with renal Fanconi syndrome: Good responses to treatment with glucocorticoids
Primary Sjogren's syndrome with renal Fanconi syndrome: Good responses to treatment with glucocorticoids
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DOI：
10.1016/j.semarthrit.2020.03.017
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发表时间：
2020-12-01
影响因子：
5
通讯作者：
Chen, Limeng
中科院分区：
文献类型：
--
作者：
Shi, Xiaoxia;Chen, Zhixin;Chen, Limeng
背景：肾范可尼综合征（FS）在原发性干燥综合征（pSS）中很少见。方法：回顾性分析25例pSS相关性FS（pSS-FS）患者的临床资料、肾脏病理和随访资料，并对治疗效果进行分析。结果：25例pSS-FS患者以女性为主（92.0%），平均年龄43.6 ± 11.3岁。他们表现出不同程度的近端肾小管功能障碍和eGFR下降（60.9 +/- 32.3 ml/min/1.73 m（2））。pSS-FS患者的肾脏病理表现为肾小管间质性肾炎伴刷状缘缺损和淋巴浆细胞浸润。糖皮质激素治疗后，eGFR水平在第三个月随访时从48.3 +/- 20.6 ml/min/1.73 m（2）显著改善至55.0 +/- 19.9 ml/min/1.73 m（2）（P = 0.012）。免疫学反应良好（90.0%），肾小管反应良好（88.2%）。结论：在pSS-FS患者中，应用糖皮质激素可改善eGFR和肾小管功能。pSS组在免疫学特征和肾脏受累方面具有特殊的模式。(C)2020爱思唯尔公司All rights reserved.
Background: Renal Fanconi syndrome (FS) is rare in primary Sjogren's syndrome (pSS). We aimed to describe the clinicopathological characteristics of pSS associated FS (pSS-FS) and its responses to treatment.Methods: We reported 25 cases of pSS-FS patients and retrospectively reviewed their clinical records, kidney pathology and follow-up data.Results: The 25 pSS-FS patients were mainly female (92.0%) and the mean age at diagnosis was 43.6 +/- 11.3 years. They showed different degrees of proximal tubular dysfunctions and eGFR decline (60.9 +/- 32.3 ml/min/1.73m(2)). Kidney pathology of pSS-FS patients showed tubulo-interstitial nephritis with defective brush border and lymphoplasma cyticinfiltrates. After glucocorticoid treatment, the eGFR levels were significantly improved from 48.3 +/- 20.6 ml/min/1.73m(2) to 55.0 +/- 19.9 ml/min/1.73m(2) (P = 0.012) at the third month of follow-up. They also acquired good tubular (88.2%) and immunological (90.0%) responses. pSS-FS patients with young-onset pSS presented with a higher prevalence of positive anti-SSB antibody and hypocomplementemia, more severe hypokalemia, and better eGFR levels.Conclusions: In pSS-FS patients, use of glucocorticoids could improve eGFR and tubular functions. The youngonset pSS group presented with a particular pattern in immunological features and kidney involvement. (C) 2020 Elsevier Inc. All rights reserved.