Retinal pigment epithelial changes in chronic Vogt-Koyanagi-Harada disease: fundus autofluorescence and spectral domain-optical coherence tomography findings

Abstract

Purpose: The purpose of this study was to determine whether fundus autofluorescence (FAF) and spectral domain-optical coherence tomography (SD-OCT) imaging allow better assessment of retinal pigment epithelium and the outer retina in subjects with chronic Vogt-Koyanagi-Harada disease compared with examination and angiography alone.

Methods: A cross-sectional analysis of a series of seven consecutive patients with chronic Vogt-Koyanagi-Harada disease undergoing FAF and SD-OCT was conducted. Chronic disease was defined as duration of intraocular inflammation >3 months. Color fundus photographs were correlated to FAF and SD-OCT images. The images were later correlated to fluorescein angiography and indocyanine green angiography.

Results: All patients had sunset glow fundus, which resulted in no apparent corresponding abnormality on FAF or SD-OCT. Lesions with decreased autofluorescence signal were observed in 11 eyes (85%), being associated with loss of the retinal pigment epithelium and involvement of the outer retina on SD-OCT. In 5 eyes (38%), some of these lesions were very subtle on clinical examination but easily detected by FAF. Lesions with increased autofluorescence signal were seen in 8 eyes (61.5%), showing variable involvement of the outer retina on SD-OCT and corresponding clinically to areas of retinal pigment epithelium proliferation and cystoid macular edema.

Conclusion: Combined use of FAF and SD-OCT imaging allowed noninvasive delineation of retinal pigment epithelium/outer retina changes in patients with chronic Vogt-Koyanagi-Harada disease, which were consistent with previous histopathologic reports. Some of these changes were not apparent on clinical examination.

Fig. 1

Right eye of a 42-year-old female with chronic Vogt-Koyanagi-Harada disease (Case 1). A. Color fundus photograph shows sunset glow fundus, a peripapillary halo and subtle hypopigmented lesions inferiorly. B. Fundus autofluorescence imaging reveals decreased autofluorescence signal of these lesions and of the peripapillary halo. Autofluorescence of the areas of sunset glow fundus is similar to that of normal areas. C. Infrared imaging of the fundus, with corresponding spectral domain optical coherence tomography (SD-OCT) image. The red arrow points to a hypopigmented lesion and the red box marks the area shown in higher magnification below. D. Intermediate-phase fluorescein angiography, revealing hyperfluorescence (window defect) of the peripapillary halo and hypopigmented lesions. E. Late-phase indocyanine green angiography revealing hypofluorescence (choriocapillaris nonperfusion) of the peripapillary halo and hypopigmented lesions. F. Magnified view of red box indicated in C. The red arrow corresponds to the hypopigmented lesion in C. Thinning of retinal pigment epithelium/Bruch’s membrane layer (RPE atrophy) is demonstrated between the black arrows. The remaining retina is preserved.

Fig. 2

Right eye of a 41-year-old female with chronic Vogt-Koyanagi-Harada disease (Case 2). A. Color fundus photograph shows sunset glow fundus inferior to the optic disc. In addition, a subtle peripapillary halo and focal lesions with variable pigmentation are seen. B. Fundus autofluorescence imaging demonstrates decreased autofluorescence of both pigmented and atrophic scars. Note normal autofluorescence in area of sunset glow fundus. C. Infra-red imaging of the fundus, with corresponding spectral domain optical coherence tomography (SD-OCT) image. The red arrows point to hyperpigmented lesions and the black arrow indicates an atrophic focus. The red box marks the area shown in higher magnification below. D. Intermediate-phase fluorescein angiography, revealing hyperfluorescence (window defect) of the peripapillary halo and hypopigmented lesions. The hyperpigmented lesions block fluorescence. E. Mid-phase indocyanine green angiography revealing hypofluorescence of atrophic lesions, peripapillary atrophic halo, and of pigmented lesions. F. Magnified view of the red box delineated in C. The red arrows indicate pigmented scars, with thickening of retinal pigment epithelium/Bruch’s membrane (RPE/BM) layer (RPE hyperplasia/hypertrophy) and disruption of outer retinal layers. The hypopigmented focus shows thinning of RPE/BM layer (RPE atrophy) and disruption of outer retinal layers.

Fig. 3

Left eye of a 41-year-old female with chronic Vogt-Koyanagi-Harada disease (Case 2). A. Color fundus photograph discloses sunset glow fundus, peripapillary halo, multiple nummular atrophic lesions and few pigmented lesions. B. Fundus autofluorescence imaging reveals decreased autofluorescence of atrophic nummular scars, pigmented scars, and peripapillary halo. Area of sunset glow fundus has normal autofluorescence. C. Infrared imaging of the fundus, with corresponding spectral domain optical coherence tomography (SD-OCT) image. The red arrows delineate the area of peripapillary atrophy shown in the SD-OCT image, while the black arrows delineate the nummular atrophic scar. The red box marks the area shown in higher magnification below. D. Intermediate-phase fluorescein angiography, revealing hyperfluorescence (window defect) of the peripapillary halo and hypopigmented lesions. Areas with hyperpigmention block fluorescence. E. Mid-phase indocyanine green angiography revealing hypofluorescence of atrophic lesions, peripapillary atrophic halo, and pigmented lesions. F. Magnified view of the red box in C. The red arrows delineate the peripapillary atrophy whereas the black arrows encompass a nummular atrophic scar. Note the corresponding thinning of retinal pigment epithelium/Bruch’s membrane layer (RPE atrophy), as well as disruption of the outer retina.

Fig. 4

Left eye of a 30-year-old female with chronic Vogt-Koyanagi-Harada disease (Case 5). A. Color fundus photograph shows extensive areas of hyperpigmentation. B. Fundus autofluorescence imaging discloses mostly increased autofluorescence signal of the pigmented areas. C. Infrared imaging of the fundus, with corresponding spectral domain optical coherence tomography (SD-OCT) image. The red arrows deliminate the hyperpigmented plaque shown in the SD-OCT image. D. Intermediate-phase fluorescein angiography, revealing hypofluorescence of the hyperpigmented areas. E. Magnified view of the area indicated by the red box in C. The area between the red arrows consists of the hyperpigmented plaque, which shows thickening of retinal pigment epithelium/Bruch’s membrane layer (RPE hyperplasia) with sparing of the outer retina.