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一例成人起病的肾小管间质性肾炎和葡萄膜炎(“TINU综合征”),伴有骶髂关节炎和爱泼斯坦-巴尔病毒感染,自发转归良好。

A case of adult-onset tubulointerstitial nephritis and uveitis ("TINU syndrome") associated with sacroileitis and Epstein-Barr virus infection with good spontaneous outcome.

作者信息

Cigni Alessandro, Soro Giovanni, Faedda Rossana, Caucci Fabrizia, Amadori Fabrizio, Manca Alessandra, Tanda Francesco, Satta Andrea E

机构信息

Istituto di Patologia Medica and the Department of Pathology, University of Sassari, Sassari, Italy.

出版信息

Am J Kidney Dis. 2003 Sep;42(3):E4-10. doi: 10.1016/s0272-6386(03)00795-9.

Abstract

The authors report a case of tubulointerstitial nephritis and uveitis (TINU syndrome) in a 48-year-old woman. The patient's ocular symptoms (relapsing bilateral uveitis) began 4 months before the renal disease was diagnosed and were treated successfully with local steroid therapy. The main baseline laboratory findings were anemia, a rapid sedimentation rate, and a decreased renal function. Urinalysis results showed mild proteinuria and some hyaline and hyaline-granular casts. Immunoglobulin (Ig) G and IgM antibodies to Epstein-Barr virus (EBV) were present. The renal biopsy showed interstitial lymphocytes and infiltration by rare plasma cells, tubular atrophy without granulomas, and slight expansion of the mesangium; electronic microscopy showed rare electron-dense deposits in the mesangium; no vascular alterations were seen, and immunofluorescent staining was uniformly negative. X-ray of the pelvis showed bilateral sacroileitis, which has been previously described in only 1 case of TINU syndrome; human leukocyte antigen B27 was negative. After 6 months without any therapy, all laboratory findings were normal; after 30 months, renal function was still normal, uveitis had not relapsed, but sacroiliac involvement was still present; EBV-viral capsid antigen (VCA) IgM were still high (28 UA/mL), and the EBV IgG titers were increased (VCA>170, EBV-nuclear antigen 108 UA/mL). This case confirms that this rare entity, originally observed in children, may occur and have a favorable spontaneous renal outcome also in the adult; EBV may play a role, as previously suggested. This is, to the authors' knowledge, the first reported case of TINU syndrome with the association of a well-documented bilateral sacroileitis.

摘要

作者报告了一例48岁女性的肾小管间质性肾炎和葡萄膜炎(TINU综合征)。患者的眼部症状(复发性双侧葡萄膜炎)在肾病确诊前4个月出现,经局部类固醇治疗成功治愈。主要的基线实验室检查结果为贫血、血沉加快和肾功能下降。尿液分析结果显示轻度蛋白尿以及一些透明管型和颗粒管型。存在针对EB病毒(EBV)的免疫球蛋白(Ig)G和IgM抗体。肾活检显示间质淋巴细胞浸润及罕见浆细胞,肾小管萎缩但无肉芽肿,系膜轻度扩张;电子显微镜检查显示系膜中有罕见的电子致密沉积物;未见血管改变,免疫荧光染色均为阴性。骨盆X光显示双侧骶髂关节炎,此前仅在1例TINU综合征中描述过;人类白细胞抗原B27阴性。在未接受任何治疗6个月后,所有实验室检查结果均正常;30个月后,肾功能仍正常,葡萄膜炎未复发,但骶髂关节受累仍然存在;EBV病毒衣壳抗原(VCA)IgM仍然很高(28 UA/mL),EBV IgG滴度升高(VCA>l70,EBV核抗原108 UA/mL)。该病例证实,这种最初在儿童中观察到的罕见疾病在成人中也可能发生,并且肾脏可能有良好的自发转归;如之前所提示的,EBV可能起作用。据作者所知,这是首例报告的伴有双侧骶髂关节炎明确关联的TINU综合征病例。

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