Background/Purpose: Uveitis in Behçet’s syndrome (BS) follows a recurrent disease course with inflammatory exacerbations causing damage in the uvea, retina and optic nerve even with treatment. Frequent attacks and posterior involvement are considered as predictors of poor visual outcome. The aim of this study is to delineate the predictors of damage in more detail using a standard screening method among a group of BS patients with long-term regular follow-up.

Methods:: Patients with uveitis who were registered in our multidisciplinary BS clinic between 1990 and 2008 were screened. Among these, 50 patients who were followed for at least 10 years, who were regularly seen in our clinic at least once in every 4 months, who did not have > Grade 2 damage at baseline, and who represented different levels of damage severity during the last visit (between Grade 0 and 5) were selected. The damage severity was graded according to a validated damage grading instrument (5=worst) specifically developed for BS uveitis (Ozyazgan et al. in preparation). One patient was later excluded because it was realized that he did not fulfill these criteria. A standard form was used for retrieving data on demographics, baseline and final visual acuities, number and localization (anterior/posterior / panuveitis) of attacks during follow-up, presence of retinal infiltration, retinal hemorrhage and hypopyon uveitis. Candidate factors for damage progression were compared between patients who had a progression in damage score and those who did not.

Results:

98 eyes of 49 patients (M:F 35:14, mean age at baseline 27±8 years, mean follow-up duration 20.9±5.5 years, mean number of visits 76.5±35.2) were evaluated. The mean visual acuity was 0.02±0.08 at baseline and 0.47±0.52 at the final visit. The mean number of attacks was 13.2±9.4. Damage grades at baseline were Grade 0 in 79, Grade 1 in 16 and Grade 2 in 3 eyes. Damage grades at final visit were Grade 0 in 15, Grade 1 in 21, Grade 2 in 32, Grade 3 in 12, Grade 4 in 10 and Grade 5 in 8 eyes. There was damage progression in 81/98 eyes at the final visit. Isolated anterior uveitis attacks were not associated with progression of damage (2.5±2.9 vs 2.8±5.5, p=0.7). Parameters that were significantly more frequent among patients with damage progression were: number of attacks (14.5±10.8 vs 23.3±12.3; p=0.008), number of posterior attacks (0.4±1.2 vs 6.5±4.9, p<0.001), number of panuveitis attacks (0.8±1.3 vs 6.6±5.0, p<0.001), number of attacks with severe vitrous opacity preventing examination of the retina (0 vs 3.2±3.8, p<0.001), retinal infiltration (0.2±0.4 vs 1.4±1.9, p<0.001) and retinal hemorrhages in the arcuate region (0.1±0.2 vs 0.7±1.4, p<0.001), and the number of hypopyon attacks (0.2±1.0 vs 0.9±1.3, p=0.019).

Conclusion: This study confirmed that the anterior uveitis attacks are not associated with progressive damage in BS, whereas posterior and panuveitis attacks, attacks causing severe vitreous opacity, retinal infiltrates and hemorrhage in the arcuate region and hypopyon attacks are important predictors of damage. Patients showing these features should be treated more aggressively.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/factors-associated-with-damage-progression-in-behcets-syndrome-uveitis/