Ultra-widefield Fluorescein Angiography and Optical Coherence Tomography Findings in Tubulointerstitial Nephritis and Uveitis Syndrome

Purpose: To report the spectrum of posterior segment findings in tubulointerstitial nephritis and uveitis syndrome (TINU) and discuss the abnormalities that can be seen on imaging.Design: Retrospective, consecutive case series.Participants: Patients with TINU and posterior segment manifestations on examination or imaging.Methods: Patients with elevated urine beta-2 microglobulin (U(32M) and a diagnosis of TINU were included if they were evaluated at the Cole Eye Institute and did not have alternative etiologies for uveitis. Electronic medical records were reviewed for abnormal findings on ultra-widefield fluorescein angiography (UWFFA) and OCT.Main Outcome Measures: Presence of peripheral vascular leakage, optic disc leakage, chorioretinal lesions, or leakage within the macula on UWFFA. For OCT findings, patients were categorized as having intraretinal fluid, epiretinal membrane, or optic nerve edema.Results: Twenty eyes from 10 patients (6 female, 4 male) with a bimodal age distribution (10-46 years and 77-83 years) were included. Eighteen of 20 eyes (90%) underwent UWFFA; 13 eyes demonstrated the presence of peripheral vascular leakage, 5 eyes showed optic disc leakage, and 6 eyes had leakage within the macula. All eyes underwent OCT imaging; 7 eyes demonstrated intraretinal fluid, 4 eyes were found to have an epiretinal membrane, and 1 eye had optic nerve edema. Six eyes lacked anterior uveitis on initial or follow-up examination but had abnormal findings on UWFFA or OCT.Conclusions: Tubulointerstitial nephritis and uveitis syndrome is under-recognized in the clinical setting. It can manifest in patients of all ages, and posterior segment involvement is not uncommon. Abnormalities may be seen on posterior segment examination or imaging in the absence of anterior segment inflammation. Tubulointerstitial nephritis and uveitis syndrome should be considered in the differential diagnosis for patients presenting with bilateral uveitis without evidence of infection or other clear etiology for intraocular inflammation. (C) 2019 by the American Academy of Ophthalmology