Sanjeevani Scienthia, Prasad Pallavi, Sharma Sourabh, Bagai Sahil, Khullar Dinesh
Nephrology, Kalinga Institute of Medical Sciences, Bhubaneswar, IND.
Nephrology, Vardhman Mahavir Medical College (VMMC) and Safdarjung Hospital, New Delhi, IND.
Cureus. 2025 Jun 21;17(6):e86494. doi: 10.7759/cureus.86494. eCollection 2025 Jun.
Introduction Renal involvement in primary Sjögren's syndrome is often underdiagnosed and overlooked. There is a lack of substantial data on the renal manifestations and outcomes in these patients. This study aims to stress the importance of thorough evaluation of renal dysfunction in individuals with Sjögren's syndrome to improve their overall health and quality of life. Methods This multicentric, retrospective study analyzed patients with renal involvement in Primary Sjögren's syndrome from 2013 to 2021. Clinical profiles of those affected by primary Sjögren's syndrome and renal involvement were examined. Patients were enrolled based on specific inclusion criteria, and relevant biochemical tests and kidney biopsy results were reviewed. One-year follow-up data was also included. Results A total of 38 patients were studied, with a strong female predominance (N=36, 94.7%) and a mean age of 41.4 ± 14.08 years. Reduced estimated glomerular filteration rate (eGFR; <60 ml/min/1.73 m²) was found in 24 patients (63%), while seven (18.4%) were diagnosed with nephrotic syndrome, another seven (18.4%) had sub-nephrotic range proteinuria, and four (10.5%) presented with microscopic hematuria. Two patients (5.2%) were already on maintenance hemodialysis when diagnosed with Sjögren's syndrome, and one patient was identified post-kidney transplant. Renal biopsies revealed various pathological findings, with chronic tubulointerstitial nephritis being the most common (N=4/11, 36.3%). Immunosuppressive therapy was given to 20 patients (56%), primarily steroids alone (N=8, 21%), while others received conservative management and supportive care. Conclusion Chronic tubulointerstitial nephritis is the most frequent form of renal involvement in Primary Sjögren's syndrome, with most cases diagnosed at advanced stages of kidney disease. This study underscores the importance of early and comprehensive renal evaluations in patients with Sjögren's syndrome, as early detection and timely treatment may help prevent progression to chronic kidney disease. Learning points Screening for renal involvement in primary Sjögren's syndrome (pSS) should include urinalysis, serum creatinine levels, and kidney biopsy when appropriate. A kidney biopsy showing characteristic findings, such as tubulointerstitial nephritis (TIN), should be considered an additional supportive criterion for the classification of pSS, as it can influence both treatment decisions and renal outcomes.
引言
原发性干燥综合征的肾脏受累情况常常未得到充分诊断和重视。目前缺乏关于这些患者肾脏表现及预后的大量数据。本研究旨在强调对干燥综合征患者进行肾功能全面评估的重要性,以改善他们的整体健康状况和生活质量。
方法
这项多中心回顾性研究分析了2013年至2021年期间原发性干燥综合征合并肾脏受累的患者。对原发性干燥综合征合并肾脏受累患者的临床资料进行了检查。根据特定的纳入标准招募患者,并回顾相关的生化检查和肾活检结果。还纳入了一年的随访数据。
结果
共研究了38例患者,女性占主导(N = 36,94.7%),平均年龄为41.4±14.08岁。24例患者(63%)估算肾小球滤过率(eGFR;<60 ml/min/1.73 m²)降低,7例(18.4%)被诊断为肾病综合征,另外7例(18.4%)有亚肾病范围蛋白尿,4例(10.5%)出现镜下血尿。2例患者(5.2%)在被诊断为干燥综合征时已在接受维持性血液透析,1例患者在肾移植后被确诊。肾活检显示了各种病理结果,其中慢性肾小管间质性肾炎最为常见(N = 4/11,36.3%)。20例患者(56%)接受了免疫抑制治疗,主要是单独使用类固醇(N = 8,21%),其他患者接受了保守治疗和支持性护理。
结论
慢性肾小管间质性肾炎是原发性干燥综合征最常见的肾脏受累形式,大多数病例在肾脏疾病晚期被诊断出来。本研究强调了对干燥综合征患者进行早期和全面肾脏评估的重要性,因为早期发现和及时治疗可能有助于预防进展为慢性肾脏病。
学习要点
原发性干燥综合征(pSS)肾脏受累的筛查应包括尿液分析、血清肌酐水平,并在适当情况下进行肾活检。肾活检显示特征性结果,如肾小管间质性肾炎(TIN),应被视为pSS分类的额外支持标准,因为它会影响治疗决策和肾脏预后。