Child Kidney Dis.  2023 Jun;27(1):40-45. 10.3339/ckd.23.007.

A case report of tubulointerstitial nephritis and uveitis syndrome in children with an unfavorable outcome

• Kong SI
• Park MH
• Cheon EJ

• KMID: 2544236
• DOI: http://doi.org/10.3339/ckd.23.007

Abstract

Tubulointerstitial nephritis and uveitis (TINU) syndrome is defined as the occurrence of tubulointerstitial nephritis and uveitis in the absence of other systemic diseases. Three pediatric cases have been reported in the Republic of Korea, and we now report a fourth case. A 15-year-old girl presented to the ophthalmology department with a 1-week history of bilateral ocular discomfort that worsened on the day of presentation with redness and pain in both eyes. She was diagnosed with bilateral uveitis, and her baseline examination revealed moderate renal dysfunction and mild proteinuria. A renal biopsy was performed and confirmed the diagnosis of TINU syndrome. She was started on steroid eye drops and a 12-week course of oral steroids at a dose of 40 mg/m²/day, which completely resolved the proteinuria and mild renal function to an estimated glomerular filtration rate of 60 mL/min/1.73 m². However, the uveitis did not improve, and despite the addition of oral methotrexate as a second-line treatment, the uveitis remains unresponsive to treatment over 21 months. Further evaluation and treatment are ongoing, and active therapeutic intervention is suggested even at a pediatric age, considering the lack of improvement in renal function and uveitis to date.

Keyword

Case reports; Child; Nephritis; Tubulointerstitial nephritis and uveitis; Uveitis

Figure

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  Fig. 1. (A) Cells and keratic precipitates on the corneal endothelium and white blood cells and flare in the anterior chamber. The thin red arrow indicates leucocytes and flare. Thick red arrow indicates keratic precipitates on the corneal endothelium. (B) Optical coherence tomography shows asymmetry of both eyes, which was caused by nerve fiber edema extending toward the fovea in the left eye and venous turgor in the left eye. OD, right eye; OS, left eye.

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  Fig. 2. Pathologic findings. (A) Glomeruli and blood vessels were found to be normal, and the interstitium was infiltrated with inflammatory cells such as lymphocytes and plasma cells, and the tubules were found to be atrophied (silver stain, ×400). Red arrows indicate atrophied tubules. (B) Plasma cells and other types of inflammatory cells are seen in the renal tubules, and the tubules are atrophied. Red arrows indicate lymphocyte infiltration of the interstitium (silver stain, ×400).

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  Fig. 3. Electron microscopy reveals inflammatory cells in the tubules and fibrosis of the interstitium. The red arrow indicates inflammatory cells in the tubules.

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  Fig. 4. Clinical course of patient over 1 year, proteinuria (A), serum creatinine (B), and eGFR (C) were significantly improved. Oral prednisolone (40 mg/kg/day) was prescribed for 12 weeks and the methotrexate of 10 mg was used. After 1 year, the dose of methotrexate was increased to 15 mg per day. Topical steroid for uveitis was used all the time. eGFR, estimated glomerular filtration rate.

Reference

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