Review Article

Prognostic Factors of Uveitis in Behcet’s Disease: A Tunisian Study Over 28 Years

Abstract

Behcet’s disease (BD) is an inflammatory disorder of unknown etiology, characterized by recurrent oral ulcers, genital ulcers, skin lesions, and ocular involvement. Ocular involvement occurs in 25 to 80% of patients. We report the data of our monocentric, retrospective study which conducted in our departement between 1996 and 2024 including patients diagnosed as BD and had uveitis confirmed by ophthalmological examination. Among the 343 patients with BD, 114 had ocular involvement, and 88 patients met the inclusion criteria for analysis. The mean age at BD diagnosis was 36,6 ± 11,34 years, while the mean age at uveitis onset was 36,2 ± 11,5 years. 36,8% were in the third decade of life. They were 67 men and 21 women. Uveitis manifested acutely in 52 patients (59%). It was the initial symptom of BD in 73 patients (61,9%). The most common symptoms were decreased visual acuity (72%), eye redness (42%), and eye pain (36,1%). Extraocular manifestations included oral aphthous ulcers (100%), genital ulcers (85,4%), necrotizing pseudofolliculitis (72,9%), a positive pathergy test (30,4%), joint involvement (28,6%), venous thrombosis (22,2%), neurological involvement (14%), and arterial involvement (7,6%). A total of 150 eyes were affected by uveitis (107 in men, 43 in women), with bilateral involvement in 67 patients (76,1%). The anatomical distribution showed that panuveitis with retinal vasculitis was the most common presentation (63 eyes, 42%), followed by isolated panuveitis (56 eyes, 37,3%). Retinal vasculitis was observed in 47 eyes, while papillitis accompanied uveitis in 34 eyes (22,7%) and retrobulbar optic neuritis in 18 eyes (12%). Fluorescein angiography, performed in 32 patients (36,4%), confirmed papillitis in 18 cases and revealed retinal vasculitis in 21 of patients examined. Initial visual assessment revealed that 36 eyes had VA < 1/10, 43 eyes (29,4%) had VA between 1/10 and 5/10, and 67 eyes (46,2%) had VA > 5/10. After treatment, the final visual outcomes showed improvement, with 23 eyes (15.9%) having a VA < 1/10, 33 eyes (22.8%) having a VA between 1/10 and 5/10, and 89 eyes (61,4%) achieving a VA > 5/10. Treatment outcomes were considered favorable in 61,4% of cases, partially favorable in 22,8% of cases, and unfavorable in 15,9% of cases. 98,8% of patients received Colchicine, 6 patients received NSAIDs, Seventy-three patients (96,1%) received systemic corticosteroid treatment. High-dose corticosteroids were administered to 64 patients and 45 patients (59,2%) received pulse therapy with methylprednisolone (1 g/day for 3 days).  Immunosuppressive therapy was added in 27 cases (35.5%), including cyclophosphamide (22 cases), azathioprine (11 cases), and cyclosporine (4 cases) after failure of azathioprine and cyclophosphamide, only 1 case received Thalidomid. Cataract was the most common complication, affecting 45,5%, followed by posterior synechiae in 15,9%, optic atrophy in 14.8%, maculopathy in 12,5% and glaucoma in 2.4%. Relapses were observed in 30 patients (34,1%), with an average of 2 relapses per patient. Univariate analysis identified this factors as associated with a poor visual prognosis including initial visual acuity < 1/10 (p = 0.000), presence of optic atrophy (p = 0.01) and occurrence of ocular relapses (p = 0.008). Multivariate analysis confirmed 3 independent prognostic factors for poor visual outcome which are: the initial visual acuity < 1/10 (OR: 5.21, 95% CI: 1.13-23.86, p = 0.034), the ocular relapses (OR: 8.39, 95% CI: 1.48-47.4, p = 0.016) and the optic atrophy (OR: 18.04, 95% CI: 0.90-358.93, p = 0.058). Univariate analysis revealed associations between complications and age, initial visual acuity < 1/10, and bilateral uveitis. Multivariate analysis identified the following independent risk factors for ocular  complications: Bilateral uveitis (OR: 4.65, 95% CI: 1.34-16.06, p = 0.015), initial visual acuity as a continuous variable (OR: 0.75, 95% CI: 0.60-0.93, p = 0.012), treatment with methylprednisolone bolus (OR: 4.53, 95% CI: 1.21-16.94, p = 0.025). Univariate analysis showed associations between relapses and initial visual acuity < 1/10 (p = 0.04) and posterior uveitis location (p = 0.041). Multivariate analysis confirmed only initial visual acuity < 1/10 as an independent risk factor for relapses (OR: 4.12, 95% CI: 1.38-12.26, p = 0.011).