Beh�et's disease in Japan and in Great Britain: a comparative study

Abstract

Behçet's disease (BD) is an important cause of visual morbidity throughout the world, but shows striking differences in racial predilection. Despite important advances in the therapeutic management of acute intraocular inflammation, the long-term impact of these new strategies on visual outcome of BD and their efficacy in different ethnic groups is unknown. A comparative study of patient characteristics, clinical ocular features and inflammatory score, and current therapy was undertaken on all patients fulfilling the International Study Group criteria for Behçet's disease and the Behçet's Disease Research Committee of Japan, who attended the Uveitis Clinics of Moorfields Eye Hospital (n=19) and Kurume University School of Medicine (KUS) (n=35) during a continuous consecutive four-week period. Japanese patients were significantly older (43.2+/-11.8 years) than the patients seen in London (35.4+/-8. 9 years). There was a predominance of male patients in both groups. All patients seen in KUS were Japanese, while the patients in London included 12 Caucasians, five Middle Eastern, one African, and one Asian. No significant differences were seen between the two populations in the duration of systemic disease or systems affected by the disease, such as mouth ulcers, genital ulcers, skin lesions including erythema nodosum, or arthritis. The duration of ocular disease was similar in both centres: around seven years. There was, however, a significant difference in the number of eyes with active anterior uveitis (59.7% KUS vs 18.4% London (chi-square: 5.4; p=0.006)) and/or posterior uveitis (31.3% KUS vs 18.4% London (chi-square: 5.42; p<0.02)). No significant differences were found in the number of eyes with optic disc swelling or optic atrophy and in each centre the number of eyes with vision greater than 6/9 or worse than 6/60 were the same. The treatment schedules were very different between the two centres. More patients were treated with topical steroids in Japan (68.7% KUS vs 10.5% London (chi-square: 30.5; p=0.001), but a similar number used concomitant intraocular pressure-lowering agents. More patients received systemic steroids in London (84.2% London vs 17% KUS (chi-square: 20.25; p<0.001)). Three patients received systemic steroids alone, five had prednisolone and cyclosporin, four had prednisolone and azathioprine, and four had triple therapy with prednisolone, cyclosporin, and azathioprine. Only one patient used colchicine. Cyclosporin use was similar in London and KUS (47.4% and 42.8%, respectively). In Japan, three patients used prednisolone alone and three tacrolimus (FK506). In addition, two patients, who were on steroids alone, took colchicine as well. More patients in Japan had undergone surgery for cataract and glaucoma (chi-square: 4.0; p=0.045). In KUS, seven of 67 eyes had cataract surgery. A further three eyes had visually significant cataract and two eyes had undergone glaucoma surgery. In contrast, no patients in London had undergone any surgery up to and including this period.

Spectrum of Behçet’s disease in the Indian population

“…The most common complications observed were cataracts and cystoid macular edema, along with raised intraocular pressure and obliterative vasculitis. These ocular findings were comparable to other reported studies from the rest of the world (Table 5) [15][16][17][18]. A higher incidence of cataracts seen in the present series could be due to a long-standing intraocular inflammation and long-term corticosteroid therapy.…”

“…While the emphasis is placed on the composition of major signs in the Japanese criteria, the presence of oral aphthae is absolutely necessary for the diagnosis of Behçet's disease according to the international criteria [5,6]. Our clinical profile was comparable to other published series as well (Table 5) [15][16][17][18].…”

“…Rohatgi and Singal [7] reported Historically, ocular involvement is seen in nearly 60-80% of the patients [13,14]. A higher preponderance of ocular involvement in the present and certain other studies [15][16][17] is probably due to the fact that all of these studies were uveitis clinic-based studies and, hence, an obvious preponderance of patients with ocular involvement is expected. This also probably explains the observation of lesser ocular involvement by Rohatgi and Singal [7], who only reported patients referred from a dermatology clinic with a proven diagnosis of Behçet's disease.…”

Male sex, erythema nodosum, and electroretinography as predictors of visual prognosis after cataract surgery in patients with Behçet disease

“…These findings differ from those in studies from the United Kingdom and Japan, in which a slightly higher preponderance of female patients was noted. 13 We also performed a study of the association of clinical parameters, including genital ulcers, skin acne, erythema nodosum, arthritis, pustules, and furuncles, with the final CDVA. The results showed that the presence of erythema nodosum was associated with a poor visual prognosis.…”

Ocular Manifestations of Late-Onset Behçet Disease

“…A retrospective study comparing patients treated in Kurume, Japan, with those in London, UK, concluded that the disease in Japan had a more severe course. However, the English group seemed to have applied a more aggressive corticosteroid and immunosuppressive treatment approach, which might have resulted in a more benign disease course in their patients [15]. In an international collaborative study of 86 cases of childhood-onset BD from 4 different countries, uveitis was reported to be less frequent than in adults but had a very severe course, especially in male patients in this age group [16].…”