A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System

ABSTRACT

Introduction/Objective

There is currently no tool available to assess the severity of damage in uveitis due to Behçet’s syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort.

Methods

A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment. YO trained a senior and general ophthalmologist with sample fundus images. BS patients who had undergone color fundus photography during their routine visits in attack-free periods were included in the study. The color fundus photos of this prospective cohort were evaluated blindly by YO and his trainees using the new grading tool. Inter and intra-observer agreement between the graders were assessed by Cohen’s kappa analysis. The evaluation of YO was considered as the gold standard.

Results

One hundred eighty-five eyes of 108 (29 F/79 M) patients with BS uveitis were graded for damage by two investigators. Their mean age was 38,58 years and their median ocular disease duration was 13 years. The gold standard and the two investigators exhibited substantial concordance with the ocular damage grading system. The inter- and intra-observer agreement were also almost perfect.

Conclusion

The newly developed ocular damage grading system enables the standardization of damage severity in BS uveitis. It is imperative to conduct internal and external validations across diverse cohorts. Furthermore, future studies should investigate its correlation with other multimodal imaging methods such as fluorescein angiography and optical coherence tomography.

KEYWORDS:

• Behçet syndrome
• fundus photography
• grading system
• ocular damage
• uveitis

Disclosure statement

Didar Ucar has received honorariums for presentations from Amgen, AbbVie and Bayer. Sinem Nihal Esatoglu has received honorariums for presentations from UCB Pharma, Celltrion, Pfizer and Merck Sharp Dohme. Gulen Hatemi has received honoraria, consulting or speaker fees from Novartis, Pfizer, Lilly and Celltrion. Emire Seyahi has received honoraria, consulting or speaker fees from Novartis, and Pfizer. No other disclosures were reported.

Author contributions

All authors contributed to the study conception and design, including material preparation, data collection and analysis. The first draft of the manuscript was written by Yilmaz Ozyazgan, Didar Ucar, Bilge Batu-Oto, Sinem Nihal Esatoglu and Emire Seyahi and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

In memoriam

Born in Gaziantep, Turkey in 1952, Prof. Yılmaz Özyazgan pursued his medical education at Ege University, graduating in 1976. Specializing in ophthalmology at Cerrahpaşa Faculty of Medicine, Istanbul, he later enhanced his expertise in uveitis at Moorfields Eye Hospital and the National Eye Institute, USA. Rising to full professorship by 1992, he dedicated over three decades to pioneering research and patient care in uveitis and Behçet’s disease. Serving as president of the Turkish Ophthalmology Association Uvea-Behçet unit, he contributed significantly to international organizations. He authored several highly cited publications and played a significant role in several studies demonstrating the efficacy of drugs such as azathioprine, and cyclosporine in Behçet’s uveitis. He mentored numerous students and assistants, leaving a lasting legacy at Cerrahpaşa Faculty of Medicine, where he worked until his retirement in 2018. Prof. Özyazgan’s enduring dedication to learning and patient care remains exemplary.

Data availability statement

The data that support the findings of this study are available on request from the corresponding author.

Data sharing statement

All data relevant to the study are included in the article or uploaded as supplementary information.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.