Abstract
Background: Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder which could induce bilateral panuveitis involving the posterior pole and peripheral fundus. Optical coherence tomography angiography (OCTA) provides several advantages over traditional fluorescence angiography for revealing pathological abnormalities of the retinal vasculature. Until recently, however, the OCTA field of view (FOV) was limited to 6 × 6 mm2 scans.Purpose: This study examined retinal vasculature and choriocapillaris abnormalities across multiple regions of the retina (15 × 9 mm2 wide field, macular, peripapillary regions) among acute and convalescent VKH patients using a novel widefield swept-source OCTA (WSS-OCTA) device and assessed correlations between imaging features and best-corrected visual acuity (BCVA).Methods: Twenty eyes of 13 VHK disease patients in the acute phase, 30 eyes of 17 patients in the convalescent phase, and 30 eyes of 15 healthy controls (HCs) were included in this study. Vascular length density (VLD) in superficial and deep vascular plexuses (SVP, DVP), vascular perfusion density (VPD) in SVP, DVP, and choriocapillaris (CC), and flow voids (FV) in CC were measured across multiple retinal regions via WSS-OCTA (PLEX Elite 9000, Carl Zeiss Meditec Inc., USA) using the 15 × 9 mm2 scan pattern centered on the fovea and quantified by ImageJ.Results: Compared to HCs, acute phase VKH patients exhibited significantly reduced SVP-VLD, SVP-VPD, and CC-VPD across multiple retinal regions (all p < 0.01). Notably, the FV area was more extensive in VKH patients, especially those in the acute phase (p < 0.01). These changes were reversed in the convalescent phase. Stepwise multiple linear regression analysis demonstrated that macular DVP-VLD and macular CC-VPD were the best predictive factors for BCVA in the acute and convalescent VKH groups.Conclusion: The wider field of SS-OCAT provides more comprehensive and detailed images of the microvasculature abnormalities characterizing VKH disease. The quantifiable and layer-specific information from OCTA allows for the identification of sensitive and specific imaging markers for prognosis and treatment guidance, highlighting WSS-OCTA as a promising modality for the clinical management of VKH disease.
Highlights
Vogt–Koyanagi–Harada (VKH) disease is a multisystemic autoimmune disorder primarily afflicting pigmented tissues
foveal avascular zone (FAZ) and Acircularity Index (AI) The FAZ was smaller in eyes of patients with acute or convalescent VKH disease compared to control eyes, while AI was smaller in acute VKH disease eyes than convalescent VKH disease or control eyes
On fluorescein fundus angiography (FFA), the retina exhibited disseminated spotted choroidal hyperfluorescence and choroidal hypofluorescence in both acute and chronic uveitic stages [35], while indocyanine green angiography (ICGA) showed hypofluorescent dark dots during initial acute VKH uveitis episodes that usually resolved after therapy [36]
Summary
Vogt–Koyanagi–Harada (VKH) disease is a multisystemic autoimmune disorder primarily afflicting pigmented tissues. Ophthalmic manifestations could involve the choroidal stroma, retinal pigment epithelium, and outer retina at the posterior pole and peripheral fundus. The clinical course of ophthalmic manifestations can be divided into four stages, prodromal, uveitic, chronic, and chronic recurrent, according to findings from multimodal ocular vascular imaging, traditional indocyanine green angiography (ICGA), fluorescein angiography (FA), and optical coherence tomography (OCT) [5]. Injection of fluorescent dye into the circulation reveals characteristic vascular patterns that provide clues to disease progression [8]. Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder which could induce bilateral panuveitis involving the posterior pole and peripheral fundus. Optical coherence tomography angiography (OCTA) provides several advantages over traditional fluorescence angiography for revealing pathological abnormalities of the retinal vasculature. Purpose: This study examined retinal vasculature and choriocapillaris abnormalities across multiple regions of the retina (15 × 9 mm wide field, macular, peripapillary regions) among acute and convalescent VKH patients using a novel widefield swept-source OCTA (WSS-OCTA) device and assessed correlations between imaging features and best-corrected visual acuity (BCVA)
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