Bilateral Serous Retinal Detachments in Early Vogt-Koyanagi-Harada Disease Leah Nolander, OD Abstract: Vogt-Koyanagi-Harada (VKH) disease is an idiopathic multisystemic autoimmune disorder that presents with ocular, integumentary, and neurologic manifestations. The following case describes a patient with bilateral serous retinal detachments and subsequent diagnosis of VKH disease. Case History A 34-year-old African American female was referred to the Eye Center of Texas for bilateral serous retinal detachments. She presented with the chief complaint of bilaterally decreased vision, worse in the left eye than the right, and new onset of floaters in both eyes occurring constantly for the past six days. She had no history of ocular diagnoses, nor had any past ocular injuries or surgeries. Her medical history was remarkable only for hypertension, though she was not taking any systemic or ocular medications. She denied any viral prodrome prior to the incidence of blurred vision, and specifically denied any tinnitus, malaise, nausea, abdominal pain and neck or back stiffness. However, she had noted the recent onset of a dull headache also starting six days prior. Pertinent findings • BCVA: 20/60 OD 20/150 OS, NIPH OU • EOMs: Smooth, full, and accurate OU • Confrontation visual fields: Full to finger count OD/OS • Pupils: Equally round and reactive (-) APD OD/OS • IOP: 12mmHg OD, 10mmHg OS by Tonopen • Gross examination: medium-sized well-demarcated hypo-pigmentation spot on the left side of the chin. • SLE: o Conjunctiva/Sclera: White and Quiet OU o Cornea: Clear (-) KPs OU o Iris: Flat and Round (-) posterior synechia OU o AC: Deep and Quiet (-) cell/flare OU • DFE: o C/D: 0.5 round OD, 0.45 round OS with distinct margins OU o Retina: multiple serous retinal detachments radiating from ONH OU o Periphery: flat and intact OU o Vitreous: Clear (-) vitreous cells OU o Documented by Optos fundus photography OD/OS • OCT: Retinal thickening with subretinal fluid in the macular region OU • Fluorescein Angiography: late stage patchy areas of hyper-fluorescence expanding out from the optic nerve, diffuse punctate hyper-fluorescent spots (starry-sky pattern) • Assessment and Plan: o Serous Retinal Detachments OU – No history of injury or past surgery. Order blood work to rule out infectious etiologies before initiating treatment. • Blood work results: o (-)ACE, (-)Bartonella, (-)HIV, (-)Lyme, (-)RPR, (-)TB gold, o (+) ANA: positive 1:80 titer – low level positive Differential Diagnoses • The leading diagnosis was VKH disease considering the patient’s ocular and integumentary manifestations: bilateral serous retinal detachments and a vitiligo Leah Nolander Eye Center of Texas Resident hypopigmentation spot apparent on the patient’s chin. The concomitant recent onset of headaches was also suspicious for neurologic manifestations of VKH. • Differential diagnoses for presentation of bilateral serous detachments include: lymphoma, sympathetic ophthalmia, Sarcoidosis, uveal effusion syndrome, posterior scleritis, and acute multifocal placoid pigment epitheliopathy (AMPPE) 3. Diagnosis and Discussion • The incidence of VKH disease in the United States is reported as between 1 and 6 per one million patients. Among all uveitis cases, VKH represents 1-4%2. • It is most common in Japanese, Native American, and Hispanic patients, though there is evidence that affected African American patients in the United States have distant Native American ancestry1. • VKH disease is characterized by inflammation of melanocyte-containing structures including the uvea, ear, and meninges2. Autoimmunity to melanin is a key component in pathogenesis of this condition. This autoimmunity manifests as the following: o Ocular: bilateral granulomatous anterior and multifocal posterior uveitis, Dalen- Fuchs nodules, vitritis, papillitis, and exudative retinal detachments3 o Integumentary: poliosis, vitiligo, and alopecia3 o Neurologic: tinnitus, headaches, malaise, nausea, abdominal pain, neck or back stiffness, and pleocytosis of the CSF. LP may be an important diagnostic tool in atypical case presentations where VKH is suspected3. Treatment and Management • The patient was started on 100mg oral Prednisone once daily for 3 days, then 80mg daily until follow up in 2 weeks, with a planned slow taper over 3-6 months. After just three days of treatment, the patient reported significant improvement in her vision. • Although spontaneous resolution of serous detachments has been documented in VKH disease, systemic corticosteroid therapy has been found to shorten the duration of the retinal detachment and prevent further systemic involvement1. o Initial episodes of uveitis are usually well managed by intravenous or oral corticosteroids. Prompt treatment with high dose steroids and slow taper is recommended for the best visual outcome3. o Recurrences do not tend to respond as readily to systemic steroids, and often require immunosuppressive agents3. References 1. Agarwal, Anita. “Inflammatory Diseases of the Retina.” Gass' Atlas of Macular Diseases. Elsevier Health Sciences, 2011. 2. Lavezzo, M.M., Sakata, V.M., Morita, C., et al. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet Journal of Rare Diseases. 2016; 11: 29. 3. Ryan, Stephen J., Srinivas, Sadda R., Hinton, David R., et al. “Vogt-Koyanagi-Harada Disease.” Retina, Elsevier Saunders, 2013, pp. 1326–1336. Conclusion - A diagnosis of VKH should be suspected in any patient with a bilateral panuveitis, and especially when in the presence of concomitant integumentary and/or neurologic manifestations. - It is important to rule out infectious etiologies before initiating treatment with oral steroids.

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