Clinical data on 1465 subjects were collected from Germany (109 cases), UK (105), Greece (120), Turkey (239), Portugal (1), Italy (177), Morocco (15), Tunisia (62), Jordan (12), Saudi Arabia (27), Iran (337), India (104), Australia (12) and Japan (197). Men and women accounted for 979 and 454 cases, respectively. The gender of 32 subjects was not recorded. The percentages of men and women were 68.3% and 31.7%. The male‐to‐female ratio was calculated as 2.15:1. The mean age of disease onset was 27.5±10.5 years (3–71 years old). Recurrent oral aphthous ulcers were reported in 94.5%, skin lesions in 69.5% and genital ulcers in 61.4% (fig 1).
Figure 1Percentages positive for ocular and extraocular lesions are shown. In this study, we have collected the data limited to patients with ocular inflammation (100%). Recurrent oral aphthous ulcers were reported in 94.5%, skin (more ...)
Type of intraocular inflammation
Intraocular inflammation was bilateral in the majority of patients (85.6% in women, 85.5% in men). Recurrence of uveitis was seen in 95.6% of women and 95.7% of men (table 1). No difference was detected between sexes in laterality and incidence of relapse of uveitis.
Table 1Type of intraocular inflammation among men and women
Anterior segment intraocular inflammation (ASII) and combined anterior and posterior segment intraocular inflammation (CAPSII, panuveitis) were seen in 10.1% and 89.9% of women (table 1), while in men only 4.6% of the patients suffered from ASII alone, and 95.4% had panuveitis/CAPSII. The percentage of CAPSII, which is thought to be more serious in terms of visual prognosis, was significantly higher in men than in women (p<0.01, table 1).
We divided the visual prognosis into two groups based on the visual acuity at their final visit. The percentage of poor vision, less than 0.1 (20/200) in the better eye, was 23.3% in total (table 2). By gender, poor visual acuity was seen in 18.9% in women but 24.8% in men (p<0.01, table 1). The frequencies of patients with reduced visual acuity by region are summarised in table 1. The patients with poor vision were reported more frequently in India, Iran and Japan compared with other countries (p<0.01, table 2). However, Japanese patients were followed up significantly longer (17.7±10.9 years) compared with patients overall (10.3±8.4 years, p<0.01). In contrast, patients with poor vision in Italy (8.8%) were significantly fewer than in other countries (p<0.01, table 2).
Table 2Summary of clinical features of Behçet's disease on a regional basis
Analysis by country
The mean age of disease onset was calculated as 27.4±10.4 (mean±SD) years old for the whole cohort. Japanese patients were the oldest (34.3±10.3 years old). In other countries, however, symptoms began younger than 30 years old. The percentage of men by country is shown in table 2. This was significantly different only in India (p<0.01, table 2).
HLA Class I was typed in 556 subjects, and HLA‐B51 was detected in 62.0% (348 subjects) of the patients. The country‐by‐country frequencies of HLA‐B51 are shown in table 2. Behçet's patients had HLA‐B51 more frequently in Greece (p<0.01) but less frequently in the UK (p<0.01) than in the whole cohort (table 2).