A study of comparison between the nationwide epidemiological survey in 1991 and previous surveys on behçet’s disease in Japan

Abstract

The 4th nationwide epidemiological survey on Beh~et disease (BD), which included all patients with BD at 1,200 hospitals selected at random from 10,081 hospitals in Japan, was carried out by the BD Research Committee of the Ministry of Health and Welfare in 1991 to examine the epidemiological features of BD in Japan by comparing with previous surveys.3,938 patients from these hospitals were examined by the Japanese diagnostic criteria of BD (JCBD) revised in 1987 and the International criteria for dassiflcation of BD (ICBD). Among these 3,938 patients, 622 patients were only suspected of having BD or dinical signs of the disease were unknown, and most of these patients were incompatible with the ICBD. So these patients were exduded from the study of epidemiological features.The average patients age has risen 7-8 years over the last 20 years and the average age of onset in both sexes increased by about 3 years from 1972 to 1991. While a decrease in the sex ratio was seen in the complete-type and the incomplete-type BD without ocular symptoms, a sustained high sex ratio was shown in incomplete-type BD with ocular symptoms. The positive rate of HLA-B51 antigen was 54.9% (men: 56.9%, women: 52.2%) significantly higher than the 15-16% in healthy subjects but it might have been gradually decreasing. Also the clinical course of BD has become too mild for prognosis. According to these epidemiological features of BD, the clinical manifestation of BD in Japan might have become the Western type of BD.

Changes in Ocular Manifestations of Behçet Disease in Korean Patients over Time: A Single-center Experience in the 1990s and 2000s

“…2,5 Data from an epidemiologic BD survey in Japan showed that the number of patients with incomplete BD had increased between 1972 and 1991, while the number of patients with complete BD was sustained. 29 The increased ratio of incomplete/complete BD in the late 2000s compared to the 1990s was statistically significant in this study and may be associated with better visual prognosis. This result is in agreement with similar data from the Japanese epidemiologic survey.…”

Erythema Nodosum-like Lesions in Behçet’s Disease: A Clinicopatho­logical Study of 26 Cases

“…However, considering the general low frequency of gastrointestinal involvement in BD (1.6% in Turkey, 12% in Germany, 10-15% in Japan) (2,17,18,20) and the lower fi-equency of eye lesions in this study, it was remarkable that 21.1 % of the patients (4 of 19 who had the EN-like lesions with vasculitis type) showed severe BD with gastrointestinal involvement. A multicentre study disclosed that, in patients with BD, male sex and a younger age at onset are associated with more severe disease (2).…”

“…The patients with these EN-type lesions were clinically characterized as being exclusively female, and to have mild cases of BD. Recent studies show that the clinical manifestations of BD tend to be milder than those previously reported in Japanese patients and the mild cases without eye lesions show a female predominance (17). The presence of the EN-type lesion may be an indicator of the mildness of BD.…”

“…Although BD is somewhat more common among females than males in Japan (malerfemale (M:F) ratio 0.7-0.9:1) (1, [16][17][18], the present study seems to be biased to include more female patients (0.37:1 for the M:F ratio). However, previous studies have suggested that EN-like lesions in BD are more commonly seen in Japanese females than males.…”

“…The other 2 patients with severe disease had both eye and other lesions. The general fi-equency of eye lesions in BD is 30% in Turkey (2), 53% in Germany (20) and 65-69% in Japan (17,18). Therefore, the present study seems to be biased towards a lower frequency of eye lesions, probably because the subjects of this study were patients evaluated in a dermatological clinic and the predominance of female patients (who have a lower frequency of eye lesions) in this study.…”