Abstract
Purpose: :
To evaluate and integrate clinical and multimodal fundus imaging (MFI) findings in Vogt-Koyanagi-Harada (VKH) disease patients with > 6 mo from disease onset; to correlate these findings with inflammatory activity, and to better understand disease pathogenesis.
Methods: :
Nineteen patients (37 eyes) underwent prospective standardized clinical and MFI evaluations (at least two, with a minimum time interval of 6-mo), which included near infrared reflectance (niR), blue-light autofluorescence (bAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and Fourier-domain optical coherence tomography (Fd-OCT). Clinical disease activity was evaluated according to SUN guidelines. Readers who were blind for patients’ clinical data analyzed the MFI data.
Results: :
Presence of a hyperreflective amorphous material in the outer choroid was observed on Fd-OCT in 33 out of 37 eyes at baseline and follow up, and it was commonly associated to overall thinning of the choroidal compartment and a hyperreflectant reticular pattern on niR. In 19 eyes, the RPE/Bruch reflective complex presented areas of focal thickening on Fd-OCT, which was coincident with a hyperreflectant granular pattern on niR. Focal thickening did not show correlation with inflammatory activity. Fragmentation or attenuation of the photoreceptors' IS/OS reflective junction layer on Fd-OCT was observed in 19 eyes at baseline, with no obvious changes documented during follow-up in 16 eyes. In 5 eyes, the choroid presented areas of thickening on Fd-OCT, assuming a convex appearance with consequent bulging of the overlying retina. Dark dots on ICGA were observed in 8 eyes at baseline with increase in density in 6 eyes during follow up. Additional inflammatory signs (increase in anterior chamber cells and/or in optic disc hyperfluorescence on FA) were observed in 5 eyes (100%) with thickened choroids on Fd-OCT and in 6 eyes (75%) with dark dots on ICGA.
Conclusions: :
Presence of hyperreflective amorphous material with variable choroidal thinning was associated to reticular pattern on niR and seems to represent a diffuse structural damage in long standing VKH disease. On the other hand, granular pattern seems to be related to focal RPE abnormality and unrelated to disease activity. Bulging of external retina may be a sign of active choroidal inflammation. MFI may contribute to the understanding of VKH disease pathogenesis, to better evaluate disease activity and to define an adequate treatment.
Keywords: uveitis-clinical/animal model • imaging/image analysis: clinical • choroid