J Korean Ophthalmol Soc.  2022 Jul;63(7):630-636. 10.3341/jkos.2022.63.7.630.

Bilateral Frosted Branch Angiitis in Anti-phospholipid Antibody Syndrome

• Lee YM
• Lee MW
• Lee YH
• Baek SK

• KMID: 2531827
• DOI: http://doi.org/10.3341/jkos.2022.63.7.630

Abstract

Purpose
We report a case of bilateral frosted branch angiitis caused by anti-phospholipid antibody syndrome.
Case summary
A 60-year-old female complained of worsening vitreous floaters and decreased visual acuity in both eyes. The initial best-corrected visual acuity (BCVA) was 0.8 in the right eye and 0.05 in the left. On slit-lamp examination, inflammatory findings were observed in the anterior chamber and vitreous body of both eyes. On fundus examination, vascular sheathing in the shape of a frosted branch was observed in the posterior pole and peripheral retina in both eyes. Optical coherence tomography indicated macular edema in the left eye. Staining and leakage of dye along the vascular sheathing were observed in both eyes with fluorescein angiography. On suspicion of panuveitis, we conducted a blood test and started eye drops and oral steroid therapy. However, vitreous inflammation, macular edema, and vascular sheathing increased; thus, we proceeded with systemic steroid therapy. We conducted blood tests at 8-week intervals; lupus anticoagulant was negative but anticardiolipin antibody and anti- ß2 glycoprotein-I antibody were positive. We diagnosed the patient with bilateral frosted branch angiitis caused by anti-phospholipid antibody syndrome. During the follow-up period, the BCVA remained steady at 0.5 in the right eye and 0.3 in the left eye, without symptom recurrence.
Conclusions
Bilateral frosted branch angiitis, a rare disease, is known to respond well to systemic steroid treatment. However, if accompanied by primary anti-phospholipid antibody syndrome, as in the case presented, it may have an atypical prognosis.

Keyword

Antiphospholipid syndrome; Frosted branch angiitis