Chevalier Agnès, Duflos Claire, Clave Stephanie, Boyer Olivia, Hogan Julien, Lahoche Annie, Decramer Stephane, Broux Françoise, Vrillon Isabelle, Allain-Launay Emma, Bacchetta Justine, Tanne Corentin, Allard Lise, Cloarec Sylvie, Pietrement Christine, Bourdat-Michel Guylhene, Djeddi Djamal, Dunand Olivier, Faudeux Camille, Nobili François, Taque Sophie, Ulinski Tim, Zaloszyc Ariane, Morin Denis, Fila Marc
Service de Néphrologie pédiatrique, Centre Hospitalier Universitaire Montpellier, centre de référence maladies rénales rares du sud-ouest SORARE, Université de Montpellier-Filière ORKiD, Montpellier, France.
Unité de Recherche Clinique et Epidémiologique, Centre Hospitalier Universitaire Montpellier, Université de Montpellier, Montpellier, France.
Kidney Int Rep. 2021 Oct 7;6(12):3045-3053. doi: 10.1016/j.ekir.2021.09.017. eCollection 2021 Dec.
Tubulointerstitial nephritis (TIN) and uveitis (TINU) syndrome is a rare disease. The renal prognosis is generally thought to be better in children with TINU syndrome than in adults. However, data are scarce. We aimed to investigate the long-term renal prognosis in a French cohort of children with TINU syndrome.
We performed a national retrospective study including 23 French pediatric nephrology centers enrolling patients with TINU syndrome diagnosed between January 2000 and December 2018.
A total of 46 patients were included (52% female, median age 13.8 years). At diagnosis of TIN, the median estimated glomerular filtration rate (eGFR) was 30.6 ml/min per 1.73 m (4.9-62.8). The median time between diagnosis of uveitis and TIN was 0.4 months (-4.1; +17.1). All patients had anterior uveitis, but 12 (29%) were asymptomatic. Nearly all patients (44 of 46) received steroid treatment, and 12 patients (26%) received a second-line therapy. At last follow-up (median 2.8 years), the median eGFR was 87.5 ml/min per 1.73 m (60.3-152.7) and <90 ml/min per 1.73 m in 20 patients.
In our study, nearly half of the patients had renal sequelae at last follow-up. Given the possible progression to chronic kidney disease, long-term monitoring of children with TINU syndrome is mandatory. Approximately a quarter of the children had asymptomatic uveitis suggesting all children presenting with TIN should undergo systematic ophthalmologic screening even in the absence of ocular signs.
肾小管间质性肾炎（TIN）合并葡萄膜炎（TINU）综合征是一种罕见疾病。一般认为，TINU综合征患儿的肾脏预后比成人更好。然而，相关数据匮乏。我们旨在调查法国一组TINU综合征患儿的长期肾脏预后。
我们开展了一项全国性回顾性研究，纳入了法国23个儿科肾脏病中心在2000年1月至2018年12月期间诊断为TINU综合征的患者。
共纳入46例患者（52%为女性，中位年龄13.8岁）。在诊断TIN时，中位估计肾小球滤过率（eGFR）为30.6 ml/（min·1.73 m²）（4.9 - 62.8）。葡萄膜炎与TIN诊断之间的中位时间为0.4个月（-4.1；+17.1）。所有患者均患有前葡萄膜炎，但12例（29%）无症状。几乎所有患者（46例中的44例）接受了类固醇治疗，12例患者（26%）接受了二线治疗。在最后一次随访时（中位时间2.8年），中位eGFR为87.5 ml/（min·1.73 m²）（范围60.3 - 152.7），20例患者的eGFR<90 ml/（min·1.73 m²）。
在我们的研究中，近一半的患者在最后一次随访时有肾脏后遗症。鉴于可能进展为慢性肾脏病，对TINU综合征患儿进行长期监测是必要的。约四分之一的儿童有无症状性葡萄膜炎，这表明所有出现TIN的儿童即使没有眼部体征也应接受系统的眼科筛查。
