Renal Prognosis in Children With Tubulointerstitial Nephritis and Uveitis Syndrome

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Chevalier, Agnès | Duflos, Claire | Clave, Stephanie | Boyer, Olivia | Hogan, Julien | Lahoche, Annie | Decramer, Stéphane | 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

Edité par CCSD ; Elsevier -

International audience. Introduction: 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.Methods: We performed a national retrospective study including 23 French pediatric nephrology centers enrolling patients with TINU syndrome diagnosed between January 2000 and December 2018.Results: 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 m2 (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 m2 (60.3-152.7) and <90 ml/min per 1.73 m2 in 20 patients.Conclusion: 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.

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