罕见的单克隆伽玛病并发的老年人与小管间质性肾炎和葡萄膜炎综合征。

IF 3.8 2区 医学 Q2 GERIATRICS & GERONTOLOGY
Jian-Hui Zhang, Jie Liu, Qian Chen, Dan-Dan Ruan, Li-Sheng Liao, Kai-Ping Lin, Xiao-Ling Zheng, Jie-Wei Luo, Li Zhang
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引用次数: 0

摘要

背景:小管间质性肾炎和葡萄膜炎(TINU)综合征在青少年女性中普遍存在,而在老年人中发病率较低。此外,尽管这两种疾病都与免疫调节有关,但同时诊断TINU综合征和单克隆伽玛病(MG)的临床实例非常罕见。在这个病例报告中,我们提出了一个罕见的单克隆伽玛病并发的老年人与TINU综合征。病例介绍:一个74岁的女性慢性双侧前葡萄膜炎和非少尿急性肾损伤发展为TINU综合征。出乎意料的是,免疫固定电泳检测证实患者为MG。她的肾脏组织学显示小管间质变性,证实TINU综合征伴MG。由于患者肾血管轻链Kappa免疫荧光显示明显阳性,但未见单克隆免疫球蛋白沉积对肾小球或小管的损害,患者倾向于单克隆γ - γ病,其意义尚不确定。患者肾功能损害主要由TINU综合征引起,经糖皮质激素治疗后,眼部症状稳定,肾功能损害改善,尿蛋白始终呈阴性。结论:老年人群发病率高,导致疾病复杂性增加。本报告强调,TINU综合征可能会因各种附加条件而复杂化,包括罕见的偶联MG。肾组织活检对严重肾损伤病例的鉴别诊断至关重要。TINU和MG患者表现出不同的临床表现和肾损害病理。这些患者的诊断需要考虑临床资料和肾脏病理改变来指导治疗计划。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare concurrence of monoclonal gammopathies in an older adult with tubulointerstitial nephritis and uveitis syndrome.

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.

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来源期刊
BMC Geriatrics
BMC Geriatrics GERIATRICS & GERONTOLOGY-
CiteScore
5.70
自引率
7.30%
发文量
873
审稿时长
20 weeks
期刊介绍: BMC Geriatrics is an open access journal publishing original peer-reviewed research articles in all aspects of the health and healthcare of older people, including the effects of healthcare systems and policies. The journal also welcomes research focused on the aging process, including cellular, genetic, and physiological processes and cognitive modifications.
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