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Diagnosis and disease evolution of uveitis: A 10-year look back

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Key Takeaways

  • Over 50% of idiopathic uveitis patient cases received a definitive diagnosis upon extended follow-up, underscoring the need for repeated investigations.
  • Similar phenotypic expressions of different uveitis causes complicate diagnosis, delaying specific therapy and increasing recurrence risk.
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(Image Credit: AdobeStock/Phushutter)

(Image Credit: AdobeStock/Phushutter)

Data from a study that analyzed the evolution of uveitis diagnoses over a decade showed that a definitive diagnosis was established in over 50% of patients initially diagnosed with idiopathic uveitis, indicating the need for repeated investigations of these patients.1 Coauthors Gunjan Sharma and Atul Arora, from the Advanced Eye Centre, Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India, reported the findings.

This is the first study to follow these cases over the long-term and to identify phenotypes likely to undergo diagnostic revision over time.

Determining the specific cause of uveitis is the key factor in the ability to starting specific therapy and decreasing recurrences with improved visual outcomes over time.2-4

What stands in the way is that the infectious, noninfectious, and masquerade causes can have similar phenotypic expression, resulting in a delay in establishing the definitive diagnosis. As a result, more than 50% of patients are considered to have idiopathic uveitis,5,6 the authors explained.

“Many patients diagnosed with idiopathic uveitis are treated for autoimmune uveitis and receive corticosteroids with or without immunosuppressive therapy and may have multiple recurrences over time. It has been shown that up to 29% of patients diagnosed with idiopathic uveitis could have a specific underlying condition that could be diagnosed by conducting the relevant investigations.5 Therefore, the recurrent nature of the disease could be attributed to a failure to provide specific therapy due to a lack of diagnosis or misdiagnosis,” they explained.

They noted, for example, that the noninfectious uveitis might be an immunogenic response to an undiagnosed infectious agent and that the phenotypic characteristics of the disease can evolve over time. This may require more invasive and advanced investigations that could aid in establishing a definitive diagnosis years after the disease onset. “Knowledge of these phenotypes could prove beneficial, enabling closer monitoring and repeated investigations during recurrences, which could potentially uncover an etiology that might manifest over an extended period,” the investigators emphasized.

Retrospective look at uveitis

The authors conducted a chart review that identified 15,000 patients with uveitis who had presented to a tertiary care institute between 1992 and 2023. Of those patients, 123 (48.78% men; mean age, 29.11 years) who completed the 10-year follow-up visit were included in the study. The authors used the uveitis registry portal, Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS),7,8 to collect data from patients who were followed up for 10 years at the same center by the same physicians to identify phenotypes of uveitis likely to receive a revised diagnosis over the years.

The analysis showed that the most common anatomic and etiologic diagnoses at presentation were, respectively, anterior (49/123, 34.96%) and idiopathic (59/123, 47.97%) uveitis.

“At the end of 10 years, anterior uveitis remained the most common anatomic diagnosis (43/123, 39.83%), while the most common etiologic diagnosis was immune-mediated uveitis (50/123, 40.65%),” the investigators reported.

They explained that an etiologic diagnosis was established in 50.85% (30/59) of patients who had been diagnosed initially as having idiopathic uveitis.

The analysis showed that at the 10-year follow-up visit, tuberculous uveitis (39/44, 88.63%) and juvenile idiopathic arthritis–associated uveitis (16/49, 32.65%) were the most common infectious and immune-mediated etiologies.

Over the course of the study, 96 (80.67%) patients had multiple episodes of ocular inflammation (mean recurrence rate, 0.386 ± 0.24 recurrences/year).

The analysis also showed that higher recurrence rates (P < .03) also were associated with anterior uveitis (P < .01), the change in etiologic diagnosis after the first year (P < .03), a positive HLA-B27 at baseline (P < .04), and the diagnosis of a systemic disease before the onset of uveitis.

The researchers concluded, “Almost half...the cases initially labelled as idiopathic may reveal a specific etiology upon extended follow-up. Through our longitudinal study using the long-term OASIS registry, we found that patients with recurrent clinical symptoms, despite treatment, as well as those exhibiting changes in anatomic presentation (eg, transitioning from anterior uveitis to panuveitis), stand to benefit from a diagnostic reassessment—even if their initial tests were negative. An accurate diagnosis is crucial, particularly for conditions as potentially sight-threatening as uveitis.”

References
  1. Sharma G, Arora A, Rojas-Carabali W, et al. A decade-long review: insights into diagnosis and disease evolution of uveitis from a single-center study. Eye (Lond). Published online April 5, 2025. doi:10.1038/s41433-025-03772-8
  2. Tsirouki T, Dastiridou A, Symeonidis C, et al. A focus on the epidemiology of uveitis. Ocul Immunol Inflamm. 2018;26(1):2-16.
  3. London NJ, Rathinam SR, Cunningham ET Jr. The epidemiology of uveitis in developing countries. Int Ophthalmol Clin. 2010;50(2):1-17.
  4. Joltikov KA, Lobo-Chan AM. Epidemiology and risk factors in non-infectious uveitis: a systematic review. Front Med (Lausanne). 2021;8:695904.
  5. Choi RY, Rivera-Grana E, Rosenbaum JT. Reclassifying idiopathic uveitis: lessons from a tertiary uveitis center. Am J Ophthalmol. 2019;198:193-199.
  6. Pandurangan S, Samanta R, Kumawat D, Sood G, Devi TS, Agrawal A. Pattern of uveitis from a tertiary eye care center in Himalayan belt of North India. Indian J Ophthalmol. 2022;70(5):1642-1647.
  7. Ng SMS, Low R, Pak C, et al; OASIS Study Group. The role of a multicentre data repository in ocular inflammation: the Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS). Eye (Lond). 2023;37(15):3084-3096.
  8. Zhang Z, Ng Ming Sheng S, Kempen JH, et al. Uveitis registries – a digital tool for patient care, education, research, and collaboration. Ocul Immunol Inflamm. 2023;31(9):1859-1869.

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