Clinical Features and Treatment Outcomes of Patients with Tubulointerstitial Nephritis and Uveitis Syndrome: A Case Series

Describe the demographic and clinical characteristics, treatment outcomes, and ocular complications in patients with tubulointerstitial nephritis and uveitis (TINU) syndrome. Retrospective study of TINU patients seen at the Foster Center for Ocular Immunology (Duke University) and Wilmer Eye Institute (Johns Hopkins School of Medicine) between January 2014–December 2023. Patients were diagnosed using the 2021 SUN Working Group criteria. 27 TINU patients, 16 (59%) males, aged 20.4 ± 15.8 (range: 7–71) years were included. The mean follow-up time was 24.3 ± 16.5 (range: 7–75) months. All tested patients had an elevated urinary β2-microglobulin. Ten (37%) patients had positive renal biopsy. Thirteen (48%) patients presented with isolated anterior uveitis, and 14 (52%) had additional intermediate/posterior segment findings in at least one eye. At baseline, all patients were managed with topical corticosteroids (TCS), with 6 (27%) patients requiring oral prednisone (PRD) and 17 (63%) steroid-sparing immunosuppressive therapy (SS-IMT). Inflammatory control was achieved in 26 (96%) patients with TCS only (n = 4), TCS+PRD (n = 5), TCS+SS-IMT (n = 6), and TCS+PRD+SS-IMT (n = 11). Thirteen of 26 patients (50%) developed disease relapse at a mean time of 8.9 ± 18.9 (range: 1–71) months. One patient was lost to follow-up. Eighteen (67%) patients developed TINU-associated ocular complications. Five eyes from 4 patients had a median decrease in visual acuity of 2 Snellen lines (range: 1–4) during follow-up. The demographic and clinical presentation of TINU is heterogeneous. Intermediate and posterior segment manifestations are common. Treatment with topical and systemic corticosteroids only may be insufficient to prevent disease relapse, with most patients requiring long-term immunosuppressive therapy.