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一名患有肾小管间质性肾炎和葡萄膜炎综合征的老年患者罕见地并发单克隆丙种球蛋白病。

A rare concurrence of monoclonal gammopathies in an older adult with tubulointerstitial nephritis and uveitis syndrome.

作者信息

Zhang Jian-Hui, Liu Jie, Chen Qian, Ruan Dan-Dan, Liao Li-Sheng, Lin Kai-Ping, Zheng Xiao-Ling, Luo Jie-Wei, Zhang Li

机构信息

Department of Traditional Chinese Medicine, Shengli Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fuzhou, China.

Department of Digestive Endoscopy, Fujian Provincial Hospital, Fuzhou, China.

出版信息

BMC Geriatr. 2025 Jul 2;25(1):472. doi: 10.1186/s12877-025-06151-w.

Abstract

BACKGROUND

Tubulointerstitial nephritis and uveitis (TINU) syndrome is prevalent among adolescent females and less prevalent among the older people. Additionally, although both disorders are linked to immune modulation, clinical instances of simultaneous diagnosis of TINU syndrome and monoclonal gammopathies (MG) are exceedingly rare. In this case report, we present a rare concurrence of monoclonal gammopathies in an older adult with TINU syndrome.

CASE PRESENTATION

A 74-year-old woman with chronic bilateral anterior uveitis and a non-oliguric acute kidney injury developed TINU syndrome. Unexpectedly, immunofixation electrophoresis testing confirmed MG in the patient. Her renal histology showed tubulointerstitial degeneration, confirming TINU syndrome with MG. The patient tended to have monoclonal gammopathy of undetermined significance because her renal vascular light chain Kappa immunofluorescence showed significant positivity but no glomerular or tubular damage from monoclonal immunoglobulin deposition. The patient's renal impairment was principally attributed to TINU syndrome, and after glucocorticoid treatment, ocular symptoms stabilized, renal impairment improved, and urine protein remained consistently negative.

CONCLUSIONS

The high incidence of disease in the old population results in increased disease complexity. This report emphasizes that TINU syndrome might be complicated by various additional conditions, including the unusual occurrence of coupled MG. A renal tissue biopsy is crucial for a differential diagnosis in cases of severe kidney injury. Patients with TINU and MG present distinct clinical manifestations and renal damage pathologies. The diagnosis of these patients requires consideration of clinical data and renal pathological alterations to guide treatment planning.

摘要

背景

肾小管间质性肾炎和葡萄膜炎(TINU)综合征在青春期女性中较为常见,在老年人中则较少见。此外,尽管这两种疾病都与免疫调节有关,但同时诊断为TINU综合征和单克隆丙种球蛋白病(MG)的临床病例极为罕见。在本病例报告中,我们呈现了一名患有TINU综合征的老年成人罕见地并发单克隆丙种球蛋白病。

病例介绍

一名74岁女性,患有慢性双侧前葡萄膜炎和非少尿性急性肾损伤,发展为TINU综合征。出乎意料的是,免疫固定电泳检测证实该患者患有MG。她的肾脏组织学显示肾小管间质变性,证实为伴有MG的TINU综合征。该患者倾向于患有意义未明的单克隆丙种球蛋白病,因为她的肾血管轻链κ免疫荧光显示显著阳性,但没有单克隆免疫球蛋白沉积导致的肾小球或肾小管损伤。患者的肾功能损害主要归因于TINU综合征,糖皮质激素治疗后,眼部症状稳定,肾功能损害改善,尿蛋白持续阴性。

结论

老年人群中疾病的高发病率导致疾病复杂性增加。本报告强调,TINU综合征可能会并发各种其他情况,包括罕见的合并MG。对于严重肾损伤病例,肾组织活检对于鉴别诊断至关重要。患有TINU和MG的患者表现出不同的临床表现和肾损伤病理。这些患者的诊断需要考虑临床数据和肾脏病理改变以指导治疗方案制定。

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