A case of frosted branch angiitis associated with retinal vein occlusion as a complication of familial Mediterranean fever

BACKGROUND: Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever and serositis caused by autosomal recessive inheritance MEFV gene mutations. It is reported that the onset of angiitis is high among patients with this disease, but no reports were found in the field of ophthalmology in Japan. In this paper, we report one case that developed from optic disc vasculitis to frosted branch angiitis associated with retinal vein occlusion. CASE: A 39 year old male. Fever, abdominal pain and chest pain were continued from childhood. In 2006, an idiopathic fever was reported with a renal disorder. Based on the results of laboratory examinations, he was diagnosed with FMF and started oral colchicines to stabilize the symptoms. In October 2007, he complained of blurry vision in his left eye that lasted for about one week prior to his visit and decided to visit our department for an examination. Visual acuity was right 1.5 and left 1.2. Although no abnormalities were found in the anterior chamber or optic media of either eye, the left eye papilla was reddish and swollen, and varicose enlargement of the retinal veins and a small retinal hemorrhage were found. Four days later, a white vascular infiltration spread to all branches of the retinal veins at the upper-half of the left eye papilla, the hemorrhage increased in the entire retina and the visual acuity decreased to 0.1. He was hospitalized and systemic administration of an antiviral agent, an antibacterial agent and a steroidal agent (prednisolone 60 mg/day) was started. Subsequently, the left eye ocular fundus findings slowly improved and he was cured 7 months later with a visual acuity of 1.0. CONCLUSION: Frosted branch angiitis may occur with systemic gene abnormalities as an underlying condition and it is important in the future to consider FMF as a causative disease.