Matsuo T, Matsuo N
Department of Ophthalmology, Okayama University Medical School, Japan.
Ophthalmology. 1997 Jun;104(6):939-44. doi: 10.1016/s0161-6420(97)30203-6.
The authors present clinical features of 18 juvenile patients with a new type of uveitis termed bilateral iridocyclitis with retinal capillaritis (BIRC).
The authors reviewed medical records of 18 consecutive patients who showed bilateral iridocyclitis with retinal capillary leakage but no systemic manifestations during an 11-year period from January 1985 to December 1995.
Twelve of the 18 patients were female and the age at onset ranged from 9 to 17 years old. All patients had many cells in the anterior chamber and anterior vitreous, together with mutton fat keratic precipitates. Fluorescein angiography showed leakage from the optic disc and retinal capillaries, mainly in the midperiphery, which corresponded to retinal cloudiness. Macular edema was minimal, and all patients maintained good vision. The inflammation responded well topical, oral, and intravenous administration of corticosteroids, the choice of which was based on the extent of retinal inflammation. Human leukocyte antigen (HLA)-DR6 and HLA-Cw7 were associated significantly with the presence of BIRC (chi square test, P < 0.0001).
Bilateral retinal capillaritis affecting capillaries in various areas of the retina and overlying retinal cloudiness with no distinct lesions are unique to these patients. Fluorescein angiography is essential for diagnosis of BIRC.
作者介绍18例患有一种新型葡萄膜炎的青少年患者的临床特征，这种新型葡萄膜炎被称为双侧虹膜睫状体炎伴视网膜毛细血管炎（BIRC）。
作者回顾了1985年1月至1995年12月这11年间18例连续患者的病历，这些患者表现为双侧虹膜睫状体炎伴视网膜毛细血管渗漏但无全身表现。
18例患者中有12例为女性，发病年龄在9至17岁之间。所有患者前房和前部玻璃体均有大量细胞，伴有羊脂状角膜后沉着物。荧光素血管造影显示视盘和视网膜毛细血管渗漏，主要在视网膜中周部，这与视网膜混浊相对应。黄斑水肿轻微，所有患者视力保持良好。炎症对局部、口服和静脉注射皮质类固醇反应良好，其选择基于视网膜炎症的程度。人类白细胞抗原（HLA）-DR6和HLA-Cw7与BIRC的存在显著相关（卡方检验，P<0.0001）。
双侧视网膜毛细血管炎累及视网膜不同区域的毛细血管，并伴有视网膜混浊但无明显病变是这些患者所特有的。荧光素血管造影对BIRC的诊断至关重要。
