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斯洛文尼亚的肾结节病和小管间质性肾炎和葡萄膜炎综合征的特征:对过去十年全国肾活检登记处和患者数据集的分析。

Characteristics of renal sarcoidosis and tubulointerstitial nephritis and uveitis syndrome in Slovenia: An analysis of the national renal biopsy registry and patient datasets in the last decade.

出版信息

Clin Nephrol. 2021;96(1):6-15. doi: 10.5414/CNP96S02.

Abstract

BACKGROUND

Sarcoidosis is characterized by granulomatous inflammation in multiple organs. Renal involvement is rare, and granulomatous tubulointerstitial nephritis (TIN) is the predominant histologic feature. TIN is also a hallmark of tubulointerstitial nephritis and uveitis (TINU) syndrome. Diagnoses of both sarcoidosis and TINU syndrome are usually made by exclusion and by combining clinical and histological findings, and often remain misdiagnosed. The aim of this retrospective study was to determine the characteristics of renal sarcoidosis and TINU syndrome in Slovenia in the last decade (2010 - 2020).

MATERIALS AND METHODS

A thorough search of the national database of renal biopsies from January 2010 to December 2020 was performed. Inclusion criteria were TIN and a clinical history of either sarcoidosis or TINU syndrome. To compare the characteristics of our cohort with others, we also reviewed the global literature reported since 2010.

RESULTS

13 patients (9 female, 4 male) were included in our study. Indications for kidney biopsy were acute kidney injury (n = 8), acute exacerbation of chronic kidney disease (n = 4), and proteinuria (n = 1). Seven patients had clinical and histological evidence of sarcoidosis, and 6 patients were classified as having TINU syndrome. All patients were treated with corticosteroids. Of the 13 patients, 11 had improved kidney function 6 months after treatment, and proteinuria decreased in 9 patients. One patient was on dialysis at the time of diagnosis and remained so thereafter.

CONCLUSION

Renal sarcoidosis and TINU syndrome are rare but important causes of kidney injury, with a favorable long-term prognosis if properly diagnosed and treated in a timely manner.

摘要

背景

结节病的特征是多器官肉芽肿性炎症。肾脏受累罕见,肉芽肿性肾小管间质性肾炎(TIN)是主要的组织学特征。TIN 也是 tubulointerstitial nephritis and uveitis (TINU) 综合征的标志。结节病和 TINU 综合征的诊断通常通过排除和结合临床及组织学发现来确定,并且常常仍然被误诊。本回顾性研究的目的是确定过去十年(2010-2020 年)在斯洛文尼亚肾活检中肾结节病和 TINU 综合征的特点。

材料和方法

对 2010 年 1 月至 2020 年 12 月的全国性肾活检数据库进行了全面检索。纳入标准为 TIN 和结节病或 TINU 综合征的临床病史。为了比较我们的队列与其他队列的特点,我们还回顾了自 2010 年以来全球文献报道的病例。

结果

我们的研究纳入了 13 名患者(9 名女性,4 名男性)。肾活检的指征是急性肾损伤(n=8)、慢性肾脏病急性加重(n=4)和蛋白尿(n=1)。7 名患者有结节病的临床和组织学证据,6 名患者被归类为 TINU 综合征。所有患者均接受了皮质激素治疗。在 13 名患者中,11 名患者在治疗后 6 个月肾功能改善,9 名患者蛋白尿减少。1 名患者在诊断时即开始透析,此后一直如此。

结论

肾结节病和 TINU 综合征虽然罕见,但却是导致肾损伤的重要原因,如果能及时正确诊断和治疗,长期预后良好。

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