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Kidney Week

Abstract: FR-PO0953

Kidney Outcomes in Pediatric Tubulointerstitial Nephritis and Uveitis Syndrome: A Single-Center Case Series

Session Information

Category: Pediatric Nephrology

  • 1800 Pediatric Nephrology

Authors

  • Singh, Sonal, University of Rochester Medical Center, Rochester, New York, United States
  • Lapin, William Blaine, Connecticut Children's Medical Center, Hartford, Connecticut, United States
  • Collinge, Janine, Children's Eye Care of Connecticut, Rocky Hill, Connecticut, United States
  • Villegas, Leonela A., Connecticut Children's Medical Center, Hartford, Connecticut, United States
Background

Tubulointerstitial Nephritis and Uveitis (TINU) syndrome is a rare, autoimmune inflammatory disorder characterized by variable kidney and ocular manifestations. Longitudinal data describing renal recovery patterns in pediatric patients remain limited, with the largest reported cohort including 110 patients over a 20-year period. We describe a single-center pediatric cohort of 15 patients over a six-year period focusing on kidney-related outcomes.

Methods

We conducted a retrospective case series of pediatric patients diagnosed with TINU at Connecticut Children’s between January 1, 2017 to January 1, 2023, with longitudinal follow-up through the most recent clinical encounter. Diagnosis was based on clinical criteria, laboratory findings, and ophthalmologic evaluation. Follow-up intervals varied across patients reflecting real-world clinical practice.

Results

Fifteen patients were included, with a median age at presentation of 10.4 years (IQR 9.2-12.9; 53% female). Median age at diagnosis was 11.7 years [IQR 9.4-13.3]. Median follow-up duration was 4.0 years [IQR: 2.5-4.4]. In 80% of cases, uveitis preceded nephritis, while nephritis preceded uveitis in 13%. Decreased estimated glomerular filtration rate at diagnosis was present in 87% of patients, and elevated β2 microglobulin in 100%. At last follow-up, 47% (7/15) achieved complete kidney remission, while 53% (8/15) had chronic kidney disease (including 13% partial remission). Chronic ocular complications occurred in 27% (4/15) of patients.

Conclusion

Pediatric TINU syndrome exhibits heterogeneous clinical presentation and kidney recovery. Despite treatment, more than half of patients developed CKD, suggesting a substantially higher long-term CKD burden than previously described in pediatric TINU literature. Early multidisciplinary collaboration between nephrology, ophthalmology, and rheumatology is essential to optimize diagnosis, treatment, and long-term kidney and ocular outcomes.