Abstract
Vogt-Koyanagi-Harada disease (VKH) and sympathetic ophthalmia (SO) are bilateral, autoimmune granulomatous intraocular inflammatory conditions with nearly identical findings on ophthalmic exam. Both can reveal serous retinal detachments, optic disc edema, vitritis, and Dalen-Fuchs nodules, but they differ in key facets such as a history of penetrating ocular injury in SO and greater systemic associations in VKH. Diagnosis is aided by multimodal imaging, and its advances are resulting in earlier diagnosis, enhanced understanding of the disease process, and improved monitoring. Ocular coherence tomography (OCT) has largely replaced other imaging modalities in the diagnosis of VKH and SO by revealing exudative retinal detachments and choroidal thickening in the acute stage and demonstrating choroidal thinning in the chronic stage. However, characteristic findings on fluorescein angiography (FA), indocyanine green angiography (ICG), fundus autofluorescence (FAF), and B-scan ultrasound are still essential for many cases. Treatment of these diseases is initially with corticosteroids with a transition to immunomodulatory drugs for long-term control. Prompt and aggressive treatment can lead to good final visual outcomes and avoid complications such as sunset glow fundus, subretinal fibrosis, and choroidal neovascularization.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Norose K, Yano A. Melanoma specific Th1 cytotoxic T lymphocyte lines in Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 1996;80(11):1002–8.
Jakobiec FA, Marboe CC, Knowles DM, Iwamoto T, Harrison W, Chang S, et al. Human sympathetic ophthalmia: an analysis of the inflammatory infiltrate by hybridoma-monoclonal antibodies, immunochemistry, and correlative electron microscopy. Ophthalmology. 1983;90:76–95.
Davis JL, Mittal KK, Freidlin V, Mellow SR, Optican DC, Palestine AG, et al. HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Ophthalmology. 1990;97:1137–42.
Beniz J, Forster DJ, Lean JS, Smith RE, Rao NA. Variations in clinical features of the Vogt-Koyanagi-Harada syndrome. Retina (Philadelphia, Pa). 1991;11(3):275–80.
Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical course, therapy, and long-term visual outcome. Arch Ophthalmol. 1991;109(5):682–7.
Rao NA, Sukavatcharin S, Tsai JH. Vogt-Koyanagi-Harada disease diagnostic criteria. Int Ophthalmol. 2007;27(2–3):195–9.
Damico FM, Kiss S, Young LH. Vogt-Koyanagi-Harada disease. Semin Ophthalmol. 2005;20(3):183–90.
Rao NA, Gupta A, Dustin L, Chee S-P, Okada AA, Khairallah M, et al. Frequency of distinguishing clinical features in Vogt-Koyanagi-Harada disease. Ophthalmology. 2010;117(3):591–9. 599.e1.
Forster DJ, Rao NA, Hill RA, Nguyen QH, Baerveldt G. Incidence and management of glaucoma in Vogt-Koyanagi-Harada syndrome. Ophtha. 1993;100(5):613–8.
Arevalo JF, Garcia RA, Al-Dhibi HA, Sanchez JG, Suarez-Tata L. Update on sympathetic ophthalmia. Middle East Afr J Ophthalmol. 2012;19(1):13–21.
Vasconcelos-Santos DV, Sohn EH, Sadda S, Rao NA. Retinal pigment epithelial changes in chronic Vogt-Koyanagi-Harada disease: fundus autofluorescence and spectral domain-optical coherence tomography findings. Retina (Philadelphia, Pa). 2010;30(1):33–41.
Nazari H, Rao NA. Resolution of subretinal fluid with systemic corticosteroid treatment in acute Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 2012;96(11):1410–4.
Maruyama Y, Kishi S. Tomographic features of serous retinal detachment in Vogt-Koyanagi-Harada syndrome. Ophthalmic Surg Lasers Imaging. 2004;35(3):239–42.
Tsujikawa A, Yamashiro K, Yamamoto K, Nonaka A, Fujihara M, Kurimoto Y. Retinal cystoid spaces in acute Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 2005;139(4):670–7.
Yamaguchi Y, Otani T, Kishi S. Tomographic features of serous retinal detachment with multilobular dye pooling in acute Vogt-Koyanagi-Harada disease. Am J Ophthalmol. 2007;144(2):260–5.
Parc C, Guenoun J-M, Dhote R, Brézin A. Optical coherence tomography in the acute and chronic phases of Vogt-Koyanagi-Harada disease. Ocul Immunol Inflamm. 2005;13(2–3):225–7.
Bordaberry MF. Vogt-Koyanagi-Harada disease: diagnosis and treatments update. Curr Opin Ophthalmol. 2010;21(6):430–5.
Ishihara K, Hangai M, Kita M, Yoshimura N. Acute Vogt-Koyanagi-Harada disease in enhanced spectral-domain optical coherence tomography. Ophthalmology. 2009;116(9):1799–807.
Lee JE, Park SW, Lee JK, Choi HY, Oum BS, Kim HW. Edema of the photoreceptor layer in Vogt-Koyanagi-Harada disease observed using high-resolution optical coherence tomography. Korean J Ophthalmol. 2009;23(2):74–9.
Park SY, Kim SM, Song Y-M, Sung J, Ham D-I. Retinal thickness and volume measured with enhanced depth imaging optical coherence tomography. Am J Ophthalmol. 2013;156(3):557–66. e2.
Branchini L, Regatieri CV, Flores-Moreno I, Baumann B, Fujimoto JG, Duker JS. Reproducibility of choroidal thickness measurements across three spectral domain optical coherence tomography systems. Ophthalmology. 2012;119(1):119–23.
Fong AHC, Li KKW, Wong D. Choroidal evaluation using enhanced depth imaging spectral-domain optical coherence tomography in Vogt-Koyanagi-Harada disease. Retina (Philadelphia, Pa). 2011;31(3):502–9.
Maruko I, Iida T, Sugano Y, Oyamada H, Sekiryu T, Fujiwara T, Spaide RF. Subfoveal choroidal thickness after treatment of Vogt-Koyanagi-Harada disease. Retina (Philadelphia, Pa). 2011;31(3):510–7.
Nakai K, Gomi F, Ikuno Y, Yasuno Y, Nouchi T, Ohguro N, Nishida K. Choroidal observations in Vogt-Koyanagi-Harada disease using high-penetration optical coherence tomography. Graefes Arch Clin Exp Ophthalmol. 2012;250(7):1089–95.
da Silva FT, Sakata VM, Nakashima A, Hirata CE, Olivalves E, Takahashi WY, et al. Enhanced depth imaging optical coherence tomography in long-standing Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 2013;97(1):70–4.
Shinoda K, Imamura Y, Matsumoto CS, Mizota A, Ando Y. Wavy and elevated retinal pigment epithelial line in optical coherence tomographic images of eyes with atypical Vogt-Koyanagi-Harada disease. Graefes Arch Clin Exp Ophthalmol. 2012;250(9):1399–402.
Takahashi H, Takase H, Ishizuka A, Miyanaga M, Kawaguchi T, Ohno-Matsui K, Mochizuki M. Choroidal thickness in convalescent vogt-koyanagi-harada disease. Retina. 2014;34(4):775–80.
Inomata H, Rao NA. Depigmented atrophic lesions in sunset glow fundi of Vogt-Koyanagi-Harada disease. Am J Ophthalmol. 2001;131(5):607–14.
Nazari H, Hariri A, Hu Z, Ouyang Y, Sadda S, Rao NA. Choroidal atrophy and loss of choriocapillaris in convalescent stage of Vogt-Koyanagi-Harada disease: in vivo documentation. J Ophthalmic Inflamm Infect. 2014;4:9.
Tagawa Y, Namba K, Mizuuchi K, Takemoto Y, Iwata D, Uno T, Fukuhara T, Hirooka K, Kitaichi N, Ohno S, Ishida S. Choroidal thickening prior to anterior recurrence in patients with Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 2016;100(4):473–7.
Arellanes-GarcÃa L, Hernández-Barrios M, Fromow-Guerra J, Cervantes-Fanning P. Fluorescein fundus angiographic findings in Vogt-Koyanagi-Harada syndrome. Int Ophthalmol. 2007;27(2–3):155–61.
Chee S-P, Jap A, Cheung CMG. The prognostic value of angiography in Vogt-Koyanagi-Harada disease. Am J Ophthalmol. 2010;150(6):888–93.
Fardeau C, Tran THC, Gharbi B, Cassoux N, Bodaghi B, Lehoang P. Retinal fluorescein and indocyanine green angiography and optical coherence tomography in successive stages of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2007;27(2–3):163–72.
Harada T, Kanbara Y, Takeuchi T, Niwa T, Majima T. Exploration of Vogt-Koyanagi-Harada syndrome by infrared choroidal angiography with indocyanine green. Eur J Ophthalmol. 1997;7(2):163–70.
Bouchenaki N, Herbort CP. Indocyanine green angiography guided management of vogt-koyanagi-harada disease. J Ophthalmic Vis Res. 2011;6(4):241–8.
Bouchenaki N, Morisod L, Herbort CP. Vogt-Koyanagi-Harada syndrome: importance of rapid diagnosis and therapeutic intervention. Klin Monatsbl Augenheilkd. 2000;216(5):290–4.
Takemoto Y, Namba K, Mizuuchi K, Iwata D, Uno T, Ohno S, Hirooka K, Hashimoto Y, Saito W, Sugiyama K, Ishida S. Choroidal circulation impairment during the anterior recurrence of Vogt-Koyanagi-Harada disease confirmed with indocyanine green angiography and laser speckle flowgraphy. Acta Ophthalmol. 2016;94(7):e629–36.
Kawaguchi T, Horie S, Bouchenaki N, Ohno-Matsui K, Mochizuki M, Herbort CP. Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2010;30(1):41–50.
Koizumi H, Maruyama K, Kinoshita S. Blue light and near-infrared fundus autofluorescence in acute Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 2010;94(11):1499–505.
Ayata A, Dogru S, Senol MG, Unal M, Ersanli D, Bilge AH. Autofluorescence findings in Vogt-Koyanagi-Harada disease. Eur J Ophthalmol. 2009;19(6):1094–7.
Heussen FM, Vasconcelos-Santos DV, Pappuru RR, Walsh AC, Rao NA, Sadda SR. Ultra-wide-field green-light (532-nm) autofluorescence imaging in chronic Vogt-Koyanagi-Harada disease. Ophthalmic Surg Lasers Imaging. 2011;42(4):272–7.
da Silva FT, Hirata CE, Olivalves E, Oyamada MK, Yamamoto JH. Fundus-based and electroretinographic strategies for stratification of late-stage Vogt-Koyanagi-Harada disease patients. Am J Ophthalmol. 2009;148(6):939–45. e3.
Yang P, Fang W, Wang L, Wen F, Wu W, Kijlstra A. Study of macular function by multifocal electroretinography in patients with Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 2008;146(5):767–71.
Sugiura S. Vogt-Koyanagi-Harada disease. Jpn J Ophthalmol. 1978;22:9–35.
Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol. 2001;131:647–52.
Kitamura M, Takami K, Kitaichi N, Namba K, Kitamei H, Kotake S, Ohno S. Comparative study of two sets of criteria for the diagnosis of Vogt-Koyanagi-Harada’s disease. Am J Ophthalmol. 2005;139(6):1080–5.
Tsai JH, Sukavatcharin S, Rao NA. Utility of lumbar puncture in diagnosis of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2007;27(2–3):189–94.
Kim LA, Khurana RN, Parikh JG, Rao NA. Melanin-laden macrophages in the CSF to diagnose Vogt-Koyanagi-Harada simulating ocular syphilis. Ocul Immunol Inflamm. 2008;16(1):59–61.
Forster DJ, Cano MR, Green RL, Rao NA. Echographic features of the Vogt-Koyanagi-Harada syndrome. Arch Ophthalmol. 1990;108(10):1421–6.
Yang P, Liu X, Zhou H, Guo W, Zhou C, Kijlstra A. Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset. Clin Exp Ophthalmol. 2011;39(7):639–47.
Aggarwal K, Agarwal A, Mahajan S, Invernizzi A, Mandadi SK, Singh R, Bansal R, Dogra MR, Gupta V, OCTA Study Group. The role of optical coherence tomography angiography in the diagnosis and management of acute Vogt-Koyanagi-Harada disease. Ocul Immunol Inflamm. 2016;20:1–12.
Aggarwal K, Agarwal A, Deokar A, Mahajan S, Singh R, Bansal R, Sharma A, Dogra MR, Gupta V, OCTA Study Group. 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. J Ophthalmic Inflamm Infect. 2017;7(1):3.
Gupta V, Gupta A, Dogra MR, Singh I. Reversible retinal changes in the acute stage of sympathetic ophthalmia seen on spectral domain optical coherence tomography. Int Ophthalmol. 2011;31(2):105–10.
Iida T, Yannuzzi LA, Spaide RF, Borodoker N, Carvalho CA, Negrao S. Cystoid macular degeneration in chronic central serous chorioretinopathy. Retina. 2003;23(1):1–7. quiz 137-8.
Behdad B, Rahmani S, Montahaei T, Soheilian R, Soheilian M. Enhanced depth imaging OCT (EDI-OCT) findings in acute phase of sympathetic ophthalmia. Int Ophthalmol. 2015;35(3):433–9.
Muakkassa NW, Witkin AJ. Spectral-domain optical coherence tomography of sympathetic ophthalmia with Dalen-Fuchs nodules. Ophthalmic Surg Lasers Imaging Retina. 2014;45(6):610–2.
Tah V, Zarranz-Ventura J, Sim DA, Keane PA, Egan CA, Patel PJ, Lee RW, Tufail A, Pavesio CE, Westcott MC. Characterization of choroidal structure in sympathetic ophthalmia using optical coherence tomography. Invest Ophthalmol Vis Sci. 2012;53(14):1156.
Damico FM, Kiss S, Young LH. Sympathetic ophthalmia. Semin Ophthalmol. 2005;20(3):191–7.
Chan RV, Seiff BD, Lincoff HA, Coleman DJ. Rapid recovery of sympathetic ophthalmia with treatment augmented by intravitreal steroids. Retina. 2006;26(2):243–7.
Sharp DC, Bell RA, Patterson E, Pinkerton RM. Sympathetic ophthalmia. Histopathologic and fluorescein angiographic correlation. Arch Ophthalmol. 1984;102(2):232–5.
Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a single centre long-term study of 40 patients from North India. Eye (Lond). 2008;22(12):1459–64.
Wang RC, Zamir E, Dugel PU, Sipperley JO, Thirkill CE, Shabatian B, Rao NA. Progressive subretinal fibrosis and blindness associated with multifocal granulomatous chorioretinitis: a variant of sympathetic ophthalmia. Ophthalmology. 2002;109(8):1527–31.
Bernasconi O, Auer C, Zografos L, Herbort CP. Indocyanine green angiographic findings in sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol. 1998;236(8):635–8.
Saatci AO, Paşa E, Söylev MF, Koçak N, Durak I, Kaynak S. Sympathetic ophthalmia and indocyanine green angiography. Arch Ophthalmol. 2004;122(10):1568–9.
Casella AM, Farah ME, Martins MC, Hasegawa A, Oguido AP. Sympathetic ophthalmia – histopathological correlation with fluorescein and indocyanine green angiography: case report. Arq Bras Oftalmol. 2008;71(6):886–9.
Moshfeghi AA, Harrison SA, Ferrone PJ. Indocyanine green angiography findings in sympathetic ophthalmia. Ophthalmic Surg Lasers Imaging. 2005;36(2):163–6.
Fleischman D, Say EA, Wright JD, Landers MB. Multimodality diagnostic imaging in a case of sympathetic ophthalmia. Ocul Immunol Inflamm. 2012;20(4):300–2.
Conflict of Interest
Jeffrey J. Tan and Narsing A. Rao declare that they have no conflict of interest.
Informed Consent
No human studies were carried out by the authors for this chapter.
Animal Studies
No animal studies were carried out by the authors for this chapter.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Tan, J.J., Rao, N.A. (2019). Vogt-Koyanagi-Harada Disease and Sympathetic Ophthalmia. In: Rao, N., Schallhorn, J., Rodger, D. (eds) Posterior Uveitis. Essentials in Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-030-03140-4_3
Download citation
DOI: https://doi.org/10.1007/978-3-030-03140-4_3
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-03139-8
Online ISBN: 978-3-030-03140-4
eBook Packages: MedicineMedicine (R0)