Posterior manifestations and longitudinal outcomes in TINU syndrome

Abstract

Purpose : In this retrospective study, we aim to describe the ocular clinical features and long-term visual outcomes of Tubulointerstitial Nephritis and Uveitis Syndrome (TINU).

Methods : Medical records (January 2005 to August 2021) from a tertiary eye care hospital were reviewed. All patients who met the diagnostic Criteria for TINU Syndrome were included. Demographic and clinical data were collected.

Results : Twelve patients (24 eyes) with TINU were included. Median age at onset of uveitis was 13.5 years (9-45). 66.6% (n=8/12) of patients were female. Median follow-up period was 29.5 months (6-89 months). All patients had bilateral anterior uveitis. 41.6% (n=5/12) of patients had biopsy-proven acute interstitial nephritis (AIN); urinary beta-2 microglobulin (Ub2M) measurements were elevated in all tested patients (n=9/9). Posterior segment findings were seen in 66.6% of eyes (n=16/24). Posterior segment findings were including papillitis 58.3% (n=14/24), retinal vasculitis 45.8% (n=11/24), vitritis 33.3%(n=8/24), macular edema 25% (n=6/24), presence of snowballs 16.6% (n=4/24), and chorioretinal lesions 8,3% (n=2/24). 14 eyes had available fluorescein angiography (FA) imaging and of those, 78.5% (n=11/14) revealed retinal capillary leakage, and 71.4% (n=10/14) revealed optic disc staining/leakage. Visual outcomes were favorable in most eyes (n=17/20) except for 3 (one had persistent ME, one with macular scar formation, and one developed amblyopia). 91.6% (n=11/12) patients received systemic treatment. Of those, 75% (n=9/12) received systemic corticosteroid treatment and/or immunomodulatory therapy (IMT), with 41.6% (n=5/12) patients requiring biologics to control intraocular inflammation (IOI). 66.6% (n=8/12) of patients achieved IOI quiescence and of those, 2 achieved drug-free remission during follow-up period. However, one patient in remission developed a recurrence 10 months after discontinuation of IMT. Figure 1 demonstrates fundus and FA findings in a representative patient.

Conclusions : Ub2M is a useful biomarker for the diagnosis of TINU syndrome. Posterior segment manifestations are not uncommon in TINU patients. Therefore, FA is critical for the management of patients with TINU. The majority of patients required systemic treatment in order to control IOI and prevent relapses.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.