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Familial occurrence of Behçet's disease
K Nishiura1, S Kotake, A Ichiishi
1Department of Ophthalmology, Hokkaido University School of Medicine, Sapporo, Japan.
Insights
Familial Behçet
Area of Science:
- Ophthalmology
- Genetics
- Immunology
Background:
- Behçet's disease is a rare multisystem inflammatory disorder.
- Familial clustering suggests a genetic component in Behçet's disease.
- Understanding familial patterns can elucidate disease mechanisms.
Purpose of the Study:
- To investigate the familial occurrence of Behçet's disease.
- To analyze clinical and immunogenetic features of familial Behçet's disease.
- To assess the prognosis in familial Behçet's disease.
Main Methods:
- Retrospective analysis of 564 Behçet's disease patients.
- Identification of familial cases and their relatives.
- Clinical data collection including ocular manifestations and disease type.
- HLA-B5 typing for familial and sporadic cases and controls.
Main Results:
- Familial occurrence identified in 18 patients from 9 families.
- Higher male-to-female ratio (3.5:1) in familial cases compared to sporadic.
- Ocular involvement (bilateral uveoretinitis) was common, with poor visual prognosis, especially in females.
- High frequency of HLA-B5 (92%) in familial patients.
Conclusions:
- Familial Behçet's disease exhibits distinct clinical and genetic features.
- Familial cases, particularly females, show severe ocular manifestations and poor prognosis.
- A strong association with HLA-B5 suggests a significant immunogenetic predisposition in familial Behçet's disease.
Abstract:
The familial occurrence of Behçet's disease in the 564 Behçet's patients treated in the Uveitis Survey Clinic of the Hokkaido University Hospital between 1967 and 1992 was investigated. Familial occurrence of Behçet's disease was found in 18 patients from 9 families. The male-to-female ratio was 3.5 in the familial cases, which was significantly higher than in the sporadic cases. Familial occurrence was most frequently seen among siblings. Most patients, including all female patients, had the complete type. They had typical ocular lesions, and exhibited bilateral uveoretinitis with poor visual prognosis, particularly the female patients. Frequency of HLA-B5 was 92% in the familial patients, which was much higher than in both the controls and the sporadic cases. It seems probable that patients with familial occurrence of this disease have a poorer prognosis with more susceptible immunogenetic backgrounds.