Comparison of the Clinical Manifestations between Acute Vogt-Koyanagi-Harada Disease and Acute Bilateral Central Serous Chorioretinopathy

Abstract

PurposeTo compare clinical, angiographic, and optical coherence tomographic characteristics between eyes with acute Vogt-Koyanagi-Harada (VKH) disease and eyes with acute bilateral central serous chorioretinopathy (CSC), and to demonstrate distinguishing features between the two diseases in confusing cases.MethodsThe medical records of 35 patients with VKH disease and 25 patients with bilateral CSC were retrospectively reviewed. Characteristics according to slit-lamp biomicroscopy, ophthalmoscopy, fundus photography, fluorescein angiography, indocyanine green angiography, and spectral-domain optical coherence tomography were compared between the two diseases.ResultsFive of 35 patients (10 of 70 eyes, 14.3%) with VKH disease were initially misdiagnosed as CSC patients, and six of 25 patients (12 of 50 eyes, 24%) with bilateral CSC were initially misdiagnosed as patients with VKH disease. Pigment epithelial detachment in CSC and optic disc hyperemia in VKH disease show the highest positive predictive values of 100% for each disease.ConclusionsOptic disc hyperemia in VKH disease and pigment epithelial detachment in bilateral CSC are the most specific clinical manifestations of each disease at initial patient presentation.

Distinguishing features of acute Vogt-Koyanagi-Harada disease and acute central serous chorioretinopathy on optical coherence tomography angiography and en face optical coherence tomography imaging

“…However, this entity may be misdiagnosed as acute CSC, particularly in cases presenting with bilateral neurosensory detachments and PEDs [7, 8]. The pathophysiology of the two conditions is different since VKH is primarily a diffuse stromal choroiditis with secondary involvement of the choriocapillaris, whereas as CSC occurs a result of choroidal vascular hyperpermeability.…”

“…In a series reported by Yang et al [16], 22% of patients with VKH were initially misdiagnosed as CSC. Another study by Shin et al [7] reports 14.3% of VKH cases misdiagnosed due to absence of cellular inflammatory reaction or typical features such as sub-retinal septae and RPE folds on EDI-OCT in these eyes.…”

“…However, VKH is an inflammatory disease while the pathogenesis of CSC is mainly attributed to choroidal vascular dysfunction [3–6]. CSC may often be misdiagnosed as VKH [7, 8], particularly when it presents with bilateral serous retinal detachment and increased choroidal thickness on enhanced depth imaging optical coherence tomography (EDI-OCT) [9]. Failure in differentiating CSC from VKH may result in inappropriate treatment with corticosteroids or delay in therapy and irreversible visual loss.…”

Vogt–Koyanagi–Harada syndrome – current perspectives

“…As the disease progresses to the acute phase, panuveitis manifests and patients commonly present with transient attacks of panuveitis or posterior uveitis with multifocal choroiditis lesions (Figure 2A), which progress to multifocal serous detachments and can coalesce into diffuse SRD (Figure 2B),41,50 resulting in reduced vision. SRD might initially be misdiagnosed as central serous chorioretinopathy (CSCR) 51. However, in the absence of ocular inflammation and other systemic features of VKHD, the presence of optic disc hyperemia and disc leakage on FFA support the diagnosis of VKHD rather than CSCR 51…”

“…However, in CSCR, visual acuity remains good, patients do not complain of ocular pain and there is no evidence of granulomatous ocular inflammation or a hot optic disc on FFA 51,130. Infectious choroiditis caused by syphilis,131 tuberculosis,132 and Bartonella henselae infection133,134 should also been considered in the differential diagnosis of VKHD.…”