BILATERAL DIFFUSE UVEAL MELANOCYTIC PROLIFERATION MISTAKEN FOR NIVOLUMAB-INDUCED VOGT–KOYANAGI–HARADA DISEASE–LIKE SYNDROME

Abstract

We present a case of bilateral diffuse uveal melanocytic proliferation that was mistaken for Vogt–Koyanagi–Harada disease–like syndrome induced by nivolumab. After worsening of the ophthalmological condition of the patient on steroid treatment, reevaluation of imaging and symptoms enabled us to diagnose the patient correctly and successfully treat him with plasmapheresis.

Abstract

Background/Purpose:

To describe a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) that was mistaken for nivolumab-induced Vogt–Koyanagi–Harada disease–like syndrome.

Methods:

We present the case of a 58-year-old white man with metastatic renal clear cell carcinoma for which he was palliatively treated with IV nivolumab immunotherapy. The patient developed subacute onset of blurry vision and gray spots in the visual fields of both eyes, macular subretinal fluid, thickening of the retinal pigment epithelium, and swollen optic nerve heads. Differential diagnosis included nivolumab-induced Vogt–Koyanagi–Harada disease–like syndrome, and the patient was initially treated with steroids, which gave no improvement. Investigation showed the development of dark-gray patches in the peripheral retina of both eyes and cataract, which prompted reevaluation of the diagnosis, deeming BDUMP most likely. The patient was successfully treated with plasmapheresis.

Results:

The initial presentation of the case was incorrectly diagnosed as nivolumab-induced Vogt–Koyanagi–Harada disease–like syndrome. On repeat studies of the patients' symptoms and imaging, we concluded we had missed signs of BDUMP.

Conclusion:

The diagnosis BDUMP was missed in the first evaluation. We present this case to discuss the similarities and differences between this disease and nivolumab-induced Vogt–Koyanagi–Harada disease–like syndrome. More importantly, we want to highlight that reevaluation of the diagnosis on worsening of a disease was key in this unusual case.