Vogt–Koyanagi–Harada disease masquerading as a lymphoproliferative process of the uvea

A 57-year-old man of Dutch-Indonesian ancestry presented with a 1-month history of bilateral iritis, optic nerve head swelling and decreased vision. He was initially seen by his local ophthalmologist and was treated with prednisolone acetate 1% drops with no improvement, and transferred to our tertiary care institution. On presentation, the patient had an acuity of 20/60 bilaterally with no evidence of anterior chamber inflammation and normal angle structures. On dilated exam, no vitritis was evident; however, optic nerve head swelling and a diffuse bilateral choroidal infiltrate with choroidal folds but without serous retinal detachment was present (figure 1A). Extra ocular symptoms included meningismus and tinitus. Serum testing was negative for cryptococcal antigen, rapid plasma reagin (RPR), human immunodeficiency virus (HIV), bartonella IgM/IgG, quantiferon gold tuberculosis and polymerase chain reaction (PCR) for herpes simplex (HSV), herpes zoster (HZV), and cytomegalovirus (CMV). Serum lysozyme and angiotensin converting enzyme (ACE) levels were normal. The lumbar puncture revealed elevated cerebrospinal fluid (CSF) protein (88 mg/dL), normal glucose and CSF lymphocytosis (253 nucleated cells with 94% lymphocytes) with normal flow cytometry. B-scan ultrasound revealed bilateral diffuse choroidal infiltrates most prominent near the ciliary body (figure 1B). The circumferential infiltrate, the choroidal folds and the lack of serous retinal detachment were suspicious for a lymphoproliferative process of the uvea. However, the infiltrate was bilateral and the human leukocyte antigen (HLA) typing revealed HLA-DRB104 which has been associated with Vogt–Koyanagi–Harada (VKH) disease. The patient was then treated with 1 g intravenous methylprednisolone q day for 3 days with a marked decrease in the circumferential infiltrate and diffuse choroidal thickening …