Nishiura K, Kotake S, Ichiishi A, Matsuda H
Department of Ophthalmology, Hokkaido University School of Medicine, Japan.
Nippon Ganka Gakkai Zasshi. 1994 Jun;98(6):604-8.
Familial occurrence of Behçet's disease was studied in the Uveitis Survey Clinic of Hokkaido University Hospital. Eighteen patients in the nine families had familial occurrence of this disease. Male to female ratio was 0.28 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 of the females, belonged to the complete type. They had typical ocular lesions and exhibited bilateral uveoretinitis with poor visual prognosis, particularly in the females. The frequency of HLA-B5 was 92% in the familial patients, which was significantly higher than in the normal controls and the sporadic cases. It is probable that patients with familial occurrence of this disease have poorer prognosis with stronger immunogenetic backgrounds.
北海道大学医院葡萄膜炎诊疗门诊对贝赫切特病的家族聚集情况进行了研究。九个家族中的18名患者有该病的家族聚集现象。家族性病例中男性与女性的比例为0.28,显著高于散发性病例。家族聚集现象在兄弟姐妹中最为常见。大多数患者,包括所有女性患者,都属于完全型。他们有典型的眼部病变,表现为双侧葡萄膜视网膜炎症,视力预后较差,尤其是女性患者。家族性患者中HLA - B5的频率为92%,显著高于正常对照组和散发性病例。家族性发病的患者可能预后较差,且具有更强的免疫遗传背景。