Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischcmic Optic Neuropathy: A Microvascular Perspective

A 68-year-old fair-skinned Indian woman presented to our hospital with a primary complaint of vision loss in her right eye, accompanied by increased light sensitivity, pain, and redness that had persisted for one month. Additionally, she reported progressive visual impairment in her left eye over the past two years, along with oral and genital lesions, and multiple joint pains, for which she had been receiving ongoing treatment. She had previously been diagnosed with Behçet's uveitis, with systemic involvement, according to the criteria outlined by an international study group on Behçet's disease. On examination, the right eye showed pan-uveitis with a large optic disc cup and significant disc pallor. The left eye had corneal decompensation and was phthisical. Fundus fluorescein angiography (FFA) detected minimal disc leakage and peripheral vascular leaks. Optical coherence tomography angiography (OCT-A) revealed increased retinal nerve fibre layer loss, large cup, and severe peri-papillary capillary dropout. Macular OCT-A showed significant capillary loss, widened inter-capillary distances, and an enlarged foveal avascular zone. Laboratory tests revealed a strongly positive antinuclear antibody (ANA) with a speckled homogenous pattern. The erythrocyte sedimentation rate (ESR) was elevated at 52 mm/hr, and C-reactive protein (CRP) was 6.12 mg/dl. The differential leukocyte count showed elevated segmented neutrophils, while other blood parameters were normal. Serology for tuberculosis (TB), HLA-B27, p-ANCA, c-ANCA, and anti-CCP was negative. Based on her clinical presentation, histopathology, and investigations, she was diagnosed with Behçet's eye disease with multi-system involvement, including gastrointestinal and joint disease, and confirmed to have right-eye ischemic optic neuropathy. She was treated with topical steroids and intravenous methylprednisolone, resulting in significant visual improvement. After eight weeks, her vision improved to 6/36, with better contrast and color vision. Mycophenolate mofetil, oral steroids, and tacrolimus were restarted. Due to poor response to oral medications, intravenous adalimumab was considered but discontinued due to side effects. At three months, her right-eye vision stabilized at 6/24, with normal color perception, improved contrast sensitivity, and stable lab results.