Bilateral iridocyclitis with retinal capillaritis (BIRC) in children

We observed 18 patients aged from 9 to 16 years who showed bilateral iridocyclitis with retinal capillaritis. Systemic signs or symptoms were consistently absent. Mutton fat corneal deposits were present. Fluorescein angiography showed dye leakge from retinal capillaries in the midperipheral fundus and occasionally in the posterior fundus. The inflammation responded favorably to topical and/or systemic corticosteroid. All the patients maintained corrected visual acuity of 1.0 or more. There were significantly high incidences of HLA DR6 and Cw7 as compared with the general Japanese population (χ² test with Yates' correction, p<0.0001). Five patients showed positive cell mediated immunity to interphotoreceptor retinoid binding protein. These characteristics showed this condition to be a new entity of uveitis in the juveniles and was tentatively named as bilateral iridocyclitis with retinal capillaritis (BIRC).