Late-onset Beh‡et's disease: demographic, clinical, and ocular features

To evaluate the demographic, clinical, and ocular features of patients with Beh double dagger et's disease (BD) who had first symptom onset after 40 years of age and fulfilled the diagnostic criteria afterwards. This retrospective study included 42 patients with initial BD symptom onset after 40 years of age who fulfilled the diagnostic criteria afterwards, according to the International Study Group for BD. Patients were divided into two groups based on the presence of ocular involvement: group 1 had uveitis and group 2 did not have uveitis. The ocular characteristics of patients in group 1 were further examined according to age groups. Group 1A included patients between 40-50 years of age, and group 1B included patients older than 50 years of age. Clinical and demographic features of uveitic and non-uveitic patients were compared. Ocular manifestations, treatment protocols, and ocular complications in uveitic patients were noted. The ocular characteristics of group 1A and group 1B were further evaluated. Twenty-six patients (61.9%) had uveitis (group 1) and 16 (39.1%) had no uveitis (group 2). There was no significant difference between groups according to sex, age at initial admission, age at the time of initial symptom of BD, and period between initial symptom and the diagnosis of BD. The most frequent initial manifestation was oral ulcer in both groups. No statistical difference between the groups was detected with regard to the mean age of onset of each symptom. Anterior uveitis (73.1%) was the most frequent type of uveitis followed by panuveitis (19.2%) and sclerouveitis (7.7%). Uveitis was bilateral in 80.8% of patients. The incidence of anterior uveitis was higher in group 1B than in group 1A (P = 0.023). Cataract was the most common ocular complication, followed by macular edema sequelae, glaucoma, optic disc paleness, and branch retinal vein occlusion. Late-onset BD usually affects both genders equally, and the prognosis of ocular involvement is usually good. The incidence of panuveitis decreases as age increases, while the incidence of anterior uveitis increases. Ocular involvement is usually bilateral and there is no correlation between gender and uveitis type.