Ocular features of Behcet's disease: An international collaborative study

Abstract

Objective: To investigate the clinical features of ocular lesions in Behçet's disease in different countries. Methods: A descriptive questionnaire survey was performed. Results: 25 eye centres in 14 countries returned questionnaires on prevalent cases in 2006. Clinical data were analysed on 1,465 patients with ocular lesions. Recurrent oral aphthous ulcers were reported in 94.5%, skin lesions in 69.5% and genital ulcers in 61.4%. Most of the patients had bilateral and recurrent intraocular inflammation. Poor visual acuity was seen in 18.9% in women, but 24.8% in men (p,0.01). Panuveitis was seen more in men than in women (p,0.01). 23% of the patients had visual acuity equal to or worse than 20/ 200 at the final visit. The patients with poor vision were more frequently in India, Iran and Japan than in other countries (p,0.01). Conclusions: We report the largest contemporary international case series of patients with ocular involvement in Behçet's disease. Panuveitis was significantly more frequent in men than women, and men tended to have a worse visual prognosis. There were some differences in the clinical pattern of Behçet's disease in different countries. Despite modern treatment, the disease still carries a poor visual prognosis with one-quarter of the patients blind. B ehçet's disease is a multisystem disorder characterised by recurrent oral aphthous ulcers, skin lesions, genital ulcers and ocular lesions. The disease often leads to blindness in severely affected individuals. It is most prevalent between the second and fourth decades of life. Behçet's disease is found predominantly between East Asia and the Mediterranean basin, and is uncommon in the American continents, Oceania and sub-Saharan Africa.1 The distribution of uveitis and intraocular inflammation may differ in different regions of the world. In Japan, Behçet's disease is one of the three most frequent diagnoses in patients with uveitis.2 The highest prevalence rate of the disease has been reported from Turkey.3 This disease is strongly associated with the major histocompatibility complex antigen HLA-B51, first reported in 1973. 4 Populations with a high prevalence of HLA-B51 lie predominantly north of the equator, spanning Japan and Western Europe between 30˚and 45˚N. 5 The frequency of ocular involvement in patients is thought to be between 50 and 70%.6-10 The characterisitic ocular feature is a relapsing uveitis, which may involve the anterior segment, posterior segment or both. The classification of the patient's uveitis is important both therapeutically and prognostically, because those lesions affecting the posterior part of the eye tend to be persistent and blinding.11 The disease may be more severe in men than in women. 12In the present study, we examined the differences in ocular features in Behçet's disease between regions and ethnic groups on a worldwide scale and retrospectively analysed the clinical features, ocular manifestations, visual outcomes and complications in more than 1400 Behçet's patients with ocular involvement. METHODSD...

Structural changes of fovea during remission of Behçet's disease as imaged by spectral domain optical coherence tomography

“…Male gender and early age at onset of uveitis have been reported to be poor prognostic factors in Behçet's uveitis. [18][19][20][21] The marked difference of the gender ratio might reflect a difference in severity of the disease between men and women.…”

Long-term efficacy of infliximab treatment and the predictors of treatment outcomes in patients with refractory uveitis associated with Behçet’s disease

“…BD-related ocular disease manifests mostly as panuveitis or posterior uveitis and is characterized by recurrent attacks of intraocular inflammation that may cause cumulative intraocular structural damage and eventually lead to blindness (2). Although corticosteroids and immunosuppressants have been used to treat refractory BD and uveitis and have substantially improved patient prognoses, these conventional therapies sometimes fail to control acute episodes of BD-related uveitis, resulting in rapid disease progression and visual impairment (2,3).…”

Correlation between elevation of serum antinuclear antibody titer and decreased therapeutic efficacy in the treatment of Behçet’s disease with infliximab

“…BD is one of the major aetiologies of endogenous uveitis in Japan [1]; however, its prevalence and clinical features vary among countries and ethnic groups [2,3]. Recurrent episodes of inflammatory ocular attacks can cause severe visual loss.…”