Purpose: To examine retrospectively the features of Behcet's disease patien
ts with familial occurrence and make a comparison between familial Behcet's
patients with ocular lesions and those without oculal- lesions.
Methods: We sent questionnaires about Behcet's disease patients with famili
al occurrence to 114 hospitals that reported treating such patients in prev
ious nationwide hospital surveys, and to 341 hospitals selected at random i
n Japan.
Results: We obtained reports on 83 Behcet's: patients with familial occurre
nce. The positive rate of HLA-B51 was 53.1%. The positive rate of HLA-B51 a
mong patients with ocular lesions was 64.0%, that in patients without ocula
r lesions was 14.3%, and that in patients with genital ulcers was 40.9%. Th
e mean birth year and onset year of the patients with ocular symptoms was s
ignificantly lower than that of the patients without ocular symptoms. Altho
ugh there was no significant difference between these two groups, parent-ch
ild involvement was more common among the patients without ocular lesions t
han in those with ocular lesions.
Conclusions: The findings of this study suggest that the number of familial
Behcet's disease patients with ocular lesions and high HLA-B51 positivity
has been decreasing recently. To elucidate the etiology of familial occurre
nce, larger scale epidemiological studies and further molecular studies of
Behcet's disease are needed. (C) 2001 Japanese Ophthalmological Society.