Tubulointerstitial nephritis and uveitis syndrome is an uncommon disease, probably underdiagnosed in clinical practice. Its aetiology and pathogenesis remain unknown. This syndrome is defined by an association of uveitis and tubulointerstitial nephritis, with no evidence of systemic disease or infection that might cause both ocular and renal inflammation. Renal and ocular manifestations may not occur simultaneously, making the diagnosis even more challenging. Treatment includes topical and oral corticosteroids. Renal involvement usually resolves spontaneously with full recovery of kidney function, however uveitis can persist or recur years after its initial presentation. We report a case of a 13-year-old girl with tubulointerstitial nephritis and uveitis syndrome.
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