Abstract
Purpose :
Behcet’s disease (BD) is a chronic, multisystem vasculitis that may result in a blinding uveitis. The introduction of TNF-α inhibitors has substantially changed the treatment of this condition, however there remains a paucity of data regarding long term visual outcomes, particularly compared with conventional immunosuppression, and ocular vs non-ocular manifestations of BD.
Methods :
Retrospective case series of patients who met the revised International Criteria for Behcet’s Disease (ICBD) and presented between 1990–2018 to the Royal Victorian Eye and Ear Hospital, Melbourne, Australia, were reviewed. Demographic, ophthalmic examination, systemic manifestations, medication use and complications were noted.
Results :
Forty-two patients (33 males, median age 30.1 years (IQR 25.7–35.5)) were observed for a median of 6.4 years (IQR 2.8–12.9). At first ocular presentation, 25 patients met the ICBD diagnostic criteria. A further 16 (98%) met the criteria during follow-up. The most common presenting BD manifestations were ocular (N=39), oral ulcers (N=25), genital ulcers (N=8), joint involvement (N=5), skin lesions (N=4) and vascular manifestations (N=1). The majority (N=28) developed ocular disease prior to non-ocular symptoms, with ocular involvement split evenly between unilateral (N=22) vs bilateral disease. Median best corrected logMAR visual acuity at presentation was 0.301 in 54 uveitic eyes; 15 of which were anterior uveitis, 11 intermediate, 2 posterior and 26 were panuveitis. During follow-up, 41 (98%) patients were treated with corticosteroids (CS), 36 (86%) with conventional disease-modifying anti-rheumatic drugs (DMARDs) and 10 (24%) were treated with biologic DMARDs. Despite treatment, visual impairment (logMAR >0.3), legal blindness (logMAR >1) and near total blindness (light or no light perception) persisted in 23, 12 and 5 eyes, respectively. Vision threatening complications included cataract (N=28), hypopyon (N=11), glaucoma (N=7), retinal vasculitis (N=28) and cystoid macula oedema (N=24).
Conclusions :
Preliminary data suggests that ocular manifestations typically occur prior to systemic disease in our cohort of patients with BD. As expected, men of working age are most commonly affected, often with sight threatening disease at initial presentation. Despite treatment with CS and CS-sparing agents, including TNF-α inhibitors, visually significant complications with permanent vision loss still occur.
This is a 2021 ARVO Annual Meeting abstract.