Abstract: FR-PO030

Tubulointerstitial Nephritis with Uveitis Syndrome: A Case Report of a Rare Syndrome

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Scott, Adam W, Boston Children's Hospital, Boston, Massachusetts, United States
  • Hand, Matthew M, Elliot Hospital, Manchester, New Hampshire, United States
  • Teot, Lisa, Boston Children's Hospital, Boston, Massachusetts, United States
  • Stein, Deborah R., Boston Children's Hospital, Boston, Massachusetts, United States
Background

Tubulointerstitial nephritis with uveitis (TINU) syndrome is a rare disorder characterized by acute interstitial nephritis (AIN) along with concurrent development of uveitis. First described in 1975, there have been few case reports in both the ophthalmology and nephrology literature since. The underlying pathogenesis and etiology remains poorly understood. We present a case of a young adolescent male with an antecedent Epstein-Barr virus (EBV) infection who presented with severe AIN who later developed uveitis. This case highlights the importance of considering TINU in the evaluation of patients with AIN, and provides a framework for the evaluation and follow-up of such patients.

Methods

A 13 year old previously healthy Caucasian male, diagnosed with EBV via positive heterophile antibody test 2 months prior, presented to the hospital with severe fatigue and unintentional weight loss. His examination revealed a non-toxic, pale-appearing young man with a normal blood pressure. Ophthalmologic examination was unremarkable on presentation. Initial evaluation revealed severe acute kidney injury, with a peak serum creatinine of 6.8 mg/dL. His urine sediment was significantly active, with abundant white blood cell casts. He had evidence of proximal tubular dysfunction with low-molecular weight proteinuria, renal glycosuria, and renal tubular acidosis. A renal biopsy demonstrated typical findings of AIN, with only minimal fibrosis, no vascular changes, and unremarkable immunofluorescence and electron microscopy. He was started on pulse-dose steroids, followed by oral steroids. After a prolonged steroid taper over about 3 months, his creatinine improved to a nadir of 1.0 mg/dL. Shortly after discontinuing steroids, he developed bilateral eye pain and conjunctivitis. Repeat ophthalmology evaluation revealed active uveitis. The patient was placed on topical steroid therapy, with little response, and then started back on oral steroids. His renal function remained acceptable, and his urinalysis quiescent.

Conclusion

This case highlights the importance of considering TINU in patients with AIN, and the need for ongoing monitoring for the development of uveitis following resolution of AIN, as uveitis may occur sequentially and remotely from the initial presentation. The case also implicates antecedent EBV infection as a possible etiology for TINU.