Unusual Case of Vogt-Koyanagi-Harada Disease Associated with SAPHO Syndrome: A Case Report

Abstract

A 66-year-old Japanese woman who was diagnosed with synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome presented with bilateral blurred vision 4 months prior to visiting our hospital. She had visited a local ophthalmology clinic first. She was diagnosed with conjunctivitis and was prescribed antibacterial eye drops. The symptoms persisted in spite of treatment. She was then referred to our hospital. At her initial visit, the visual acuities were 0.6 in both eyes. A slit-lamp examination revealed bilateral shallow anterior chamber, and intraocular pressures of 18 mm Hg in the right eye and 16 mm Hg in the left eye. There were no cells in the anterior chamber. Fundus examination revealed bilateral annular choroidal detachment and serous retinal detachment. Fluorescein angiography showed leakage of dye from the retinal pigment epithelium (RPE) and indocyanine green angiography showed focal choroidal hypoperfusion. Optical coherence tomography showed wavy RPE line and blurry thick choroid. Systemic investigation by the physician demonstrated bilateral pleural effusions of unknown origin. The patient had a past history of breast cancer; however, no metastasis was identified via malignant cells through cytology, laboratory findings, radiographs, CT, and MRI. After the diagnosis of Vogt-Koyanagi-Harada (VKH) disease was made, the patient was treated with local and systemic steroid including high-dose intravenous corticosteroids, and 150 mg of cyclosporine per day. Seventy days after the second high-dose of intravenous corticosteroids, these medications brought a complete resolution of both choroidal and retinal detachment. VKH disease associated with SAPHO syndrome is rare. The combination of immunosuppressive drug and steroid might be helpful for severe cases of VKH disease.

Posterior sub-Tenon triamcinolone acetonide injection for annular choroidal detachment resistant to systemic immunosuppressive treatment

“…Choroidal inflammation, which causes hyperpermeability of the choriocapillaris results in fluid and albumin extravasation into the extravascular space and finally into the suprachoroidal space in VKH disease, posterior scleritis, and sympathetic ophthalmia [9] . Additionally, a thickened choroid layer may cause mechanical compression of the vortex veins, followed by choroidal outflow obstruction, increased extravascular fluid retention, and choroidal detachment [10] ; dilated choroidal veins observed on UWF indocyanine green angiography in previous studies, as well as in the current study, support this conclusion [2] . UES is a diagnosis of exclusion; therefore, other causes of annular choroidal effusion and serous retinal detachment, including VKH disease, posterior scleritis, sympathetic ophthalmia, and tumor metastasis should initially be ruled out.…”

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome may present with extra‐articular and cutaneous manifestations

“…Additionally, other ocular manifestations such as scleritis, retinal vasculitis, and blurred vision. [19][20][21]…”

“…reported two cases of uveitis and one case of recurrent uveitis in a cohort of 41 patients diagnosed with SAPHO syndrome. Additionally, other ocular manifestations such as scleritis, retinal vasculitis, and blurred vision 19–21 …”

Update on ocular manifestations of the main monogenic and polygenic autoinflammatory diseases

“…Ocular involvement as retinal vasculitis, optic neuropathy, central retinal artery occlusion and orbital inflammation has been reported in patients with CRMO (139)(140)(141). In patients with SAPHO syndrome, eye inflammatory lesions may occur as anterior scleritis, anterior uveitis, retinal vasculitis and Vogt-Koyanagi-Harada disease (142)(143)(144)(145)(146).…”