Adalimumab for Treatment of Idiopathic Frosted Branch Angiitis: A Case Report
A 14-year-old boy, who was otherwise healthy, was referred to the uveitis clinic for progressive blurring of vision in both eyes since 1 month before.\nOn examination, visual acuity was 20/400 in both eyes. Anterior segment examination revealed mild ciliary injection and anterior chamber reaction (2+ cells) but no keratic precipitates or posterior synechiae. Both eyes had inflammatory reaction in the vitreous (2+ cells) but there were no snow balls or snow bank formation. Fundus examination disclosed extensive retinal vascular sheathing and scattered intraretinal hemorrhages with severe macular edema in both eyes ().\nSystemic work-up and investigations, including a complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) titer, angiotensin converting enzyme (ACE) titer, tuberculin skin test (TST), urine analysis and chest X-ray were unremarkable. Rheumatologic tests including antinuclear antibodies (ANA), anti double strand DNA antibody (anti ds-DNA), rheumatic factor (RF), antiphospholipid antibody, anti-neutrophil cytoplasmic antibodies (ANCAs), and cryoglobulin assay were all negative. Blood CD4 and CD8 counts were within normal range and serology for the human immunodeficiency virus was also negative. Brain magnetic resonance imaging (MRI) did not show any abnormality. Blood serology for possible infectious causes including herpes family viruses, i.e. herpes simplex virus (HSV) type 1 and 2, varicella zoster virus (VZV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV), hepatitis B and C viruses, treponema, borrelia, and toxoplasma were checked. Immunoglobulin G (IgG) levels against HSV type 2 and CMV (but not IgM) were elevated. We tested the aqueous specimen after an anterior chamber paracentesis to investigate the presence of deoxyribonucleic acid (DNA) of HSV type 1 and CMV viruses. The result of polymerase chain reaction (PCR) on aqueous samples was negative for both viruses.\nUntil the results of blood tests and aqueous PCR became ready, vision continued to deteriorate down to 20/1000 and 20/800 in the right and left eyes, respectively.\nBased on the inconclusive work-up a diagnosis of idiopathic FBA was entertained and a course of systemic steroids was suggested. At this time, the patient’s parents mentioned that two years ago, for treatment of a similar condition, oral prednisolone 1 mg/kg had been commenced but soon after initiation, the patient developed severe mood changes which persisted long after discontinuation of treatment. The parents therefore rejected corticosteroid treatment. The progressive nature of the condition mandated considering other therapeutic measures to prevent macular scarring. Due to the safety profile of anti-tumor necrosis factor (TNF) agents and their rapid onset of effect, adalimumab was considered as an alternative to conventional treatment for which the parents provided written informed consent. A single dose of 40 mg pre-filled adalimumab (HUMIRA®, Abbott Laboratories, North Chicago, IL, USA) was injected subcutaneously in the patient’s right thigh. No local or systemic adverse effect was noted at the time of injection or later on.\nThree weeks after injection, visual acuity improved to 20/200 and 20/120 in the right and left eyes, respectively. Anterior chamber and vitreous reaction decreased to 1+ cell, and vascular sheathing and macular edema resolved dramatically. Repeat fluorescein angiography and optical coherence tomography (OCT) confirmed the improvement (Figs. and ).\n10 weeks after injection, visual acuity improved to 20/40 and 20/30 in the right and left eyes, respectively. In both eyes, the anterior chambers were quiet and the vitreous had only trace cellular reaction. Clinical improvement continued up to the last visit (6 months after the injection) when visual acuity reached 20/25 in both eyes with no sign of active inflammation.