Vogt-Koyanagi-Harada Disease Workup

  • Author: R Christopher Walton, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 30, 2012
 

Laboratory Studies

  • The diagnosis of VKH disease is based upon a constellation of clinical signs and symptoms with no confirmatory tests. However, several diagnostic procedures may be useful in establishing the diagnosis, including fluorescein angiography, ultrasonography, examination of the CSF, magnetic resonance imaging, and electrophysiologic testing.
  • Examination of the CSF: This test is not necessary in typical cases of VKH disease but may be useful in cases with atypical manifestations. More than 80% of patients with VKH disease exhibit a transient CSF pleocytosis consisting primarily of lymphocytes during the first several weeks of the disease. The pleocytosis resolves within 8 weeks of onset in most patients.
  • HLA typing: Although a number of HLA associations with VKH disease have been documented, HLA typing is not diagnostic of the syndrome and is not routinely recommended.
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Imaging Studies

  • Fluorescein angiography
    • Acute VKH disease: Multiple pinpoint areas of leakage at the level of the RPE overlying areas of choroiditis are visible during the arteriovenous phase. Peripapillary pinpoint hyperfluorescence may be seen if angiography is performed early in the disease course.[13] In the early and mid phases of the angiogram, radial folds of the choroid may be visible as alternating dark and light bands of fluorescence. During the later phases of the angiogram, the pinpoint areas gradually enlarge and stain the adjacent subretinal and sub-RPE fluid. Multiple serous retinal detachments with pooling of dye often are seen in the late phases of the study. Other less common findings include retinal vascular leakage and optic disc staining.
    • Recovery phase of VKH disease (after treatment with systemic corticosteroids): Most of the acute phase abnormalities, including exudative retinal detachment and disc edema, resolve during this period. Fluorescein angiography may show persistent pinpoint areas of leakage and disc staining. Some patients may exhibit window defects and areas of mottled background hyperfluorescence.
    • Chronic VKH disease: This is characterized clinically by depigmentation of the choroid. With angiography, signs of RPE atrophy are visible, such as a moth-eaten appearance, multiple window defects, and areas of alternating hyperfluorescence and hypofluorescence. Additional findings include choroidal neovascularization, retinochoroidal and arteriovenous anastomoses, and neovascularization of the disc. Macular edema is rare in this disorder but may be seen in the chronic phase. Fluorescein angiography of the left eye in a patieFluorescein angiography of the left eye in a patient with Vogt-Koyanagi-Harada disease. Mid phase is shown on the left with multiple areas of hyperfluorescence at the level of the retinal pigment epithelium. Late phase of the same angiogram (right) reveals multiple placoid areas of hyperfluorescence at the level of the retinal pigment epithelium and pooling of dye in the areas of serous detachment.
  • Optical coherence tomography (OCT): Serous retinal detachments with subretinal septa may be visible, especially early in the disease. OCT may be useful to monitor serous detachments and response to therapy. Enhanced depth imaging OCT has revealed a markedly thickened choroid in patients with active VKH.[14]
  • Indocyanine green angiography: This probably has limited prognostic value regarding outcomes.{{Ref15} However, a recent report suggests that indocyanine green angiography may be useful for monitoring choroidal inflammation and the response to therapy.[16]
    • Acute VKH disease: Indocyanine green angiography findings include delay of choriocapillaris perfusion as well as fuzzy and indistinct choroidal vessels, multiple hypofluorescent dark spots during the intermediate and late phase, and disc hyperfluorescence during the late phase.
    • Recovery phase of VKH disease (after treatment with systemic corticosteroids): Most of the acute abnormalities resolve during this period. However, some of the hypofluorescent dark spots may persist for weeks.
    • Chronic VKH disease: Findings include hypofluorescent areas during the intermediate and late phases.
  • Ultrasonography
    • The most characteristic finding is diffuse, low-to-medium reflective thickening of the posterior choroid.
    • Additional findings include serous retinal detachments, mild thickening of the sclera and/or episclera adjacent to areas of choroidal thickening, and mild vitreous opacities.
    • These ultrasonography features may be useful in monitoring the response to therapy.Patient with progressive dysacusis and recent onsePatient with progressive dysacusis and recent onset of visual loss. Fundus photo shows a large multifocal serous detachment of the right eye. B-scan ultrasonography reveals posterior choroidal thickening with an overlying retinal detachment. Patient with progressive dysacusis and recent onsePatient with progressive dysacusis and recent onset of visual loss is shown here following 6 weeks of systemic corticosteroid therapy. Diffuse depigmentation of the choroid with retinal pigment epithelium migration is seen. Residual retinal striae are present in the peripapillary region. B-scan ultrasonography shows resolution of retinal detachment and choroidal thickening.
  • MRI: This imaging study may be useful in discriminating the sclera from the choroid and retina. With T1- and T2-weighted images, the sclera is hypointense and allows differentiation between VKH disease and posterior scleritis. During the active phase of the disease, the choroid is thickened visibly and enhances following administration of gadolinium.
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Other Tests

  • Electrophysiologic testing demonstrates nonspecific abnormalities in VKH disease; however, they may be useful in monitoring the progression of the disease.
    • Electroretinogram (ERG): During the early stages of the disease, the a and b wave amplitudes of the ERG may be depressed mildly. This may persist for extended periods but often recover to near normal levels during the chronic stages of the disease.
    • Electro-oculogram (EOG): During the early stages of the diseases, the light peak of the EOG may be depressed but returns toward normal with recovery and the chronic stages of the diseases.
  • Audiologic testing
    • Many patients experience some degree of sensorineural hearing loss.
    • If possible, all patients with auditory symptoms should undergo audiologic testing.[17]
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Histologic Findings

In most cases, histopathology has shown nongranulomatous inflammation with a plasma cell infiltrate of the uvea. Lymphocytes, multinucleated giant cells, and epithelioid cells have been described in the uvea of patients with VKH disease.

Many of the giant cells and epithelioid cells contain melanin pigment. In many cases, the choriocapillaris is not involved in the inflammatory process; however, in eyes with chronic disease, inflammation may be seen in the choriocapillaris and retina. Also, in eyes with chronic VKH disease, there is loss of choroidal melanocytes.

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Contributor Information and Disclosures
Author

R Christopher Walton, MD  Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

John D Sheppard Jr, MD, MMSc  Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Andreoli CM, Foster CS. Vogt-Koyanagi-Harada disease. Int Ophthalmol Clin. 2006;46(2):111-22. [Medline].

  2. Rajendram R, Evans M, Rao NA. Vogt-Koyanagi-Harada disease. Int Ophthalmol Clin. 2005;45(2):115-34. [Medline].

  3. Yang P, Ren Y, Li B, Fang W, Meng Q, Kijlstra A. Clinical characteristics of Vogt-Koyanagi-Harada syndrome in Chinese patients. Ophthalmology. Mar 2007;114(3):606-14. [Medline].

  4. Usui Y, Goto H, Sakai JI, Takeuchi M, Usui M, Rao NA. Presumed Vogt-Koyanagi-Harada disease with unilateral ocular involvement: report of three cases. Graefes Arch Clin Exp Ophthalmol. Mar 24 2009;[Medline].

  5. da Silva FT, Damico FM, Marin ML, Goldberg AC, Hirata CE, Takiuti PH, et al. Revised diagnostic criteria for vogt-koyanagi-harada disease: considerations on the different disease categories. Am J Ophthalmol. Feb 2009;147(2):339-345.e5. [Medline].

  6. Yamaki K, Gocho K, Hayakawa K, Kondo I, Sakuragi S. Tyrosinase family proteins are antigens specific to Vogt-Koyanagi-Harada disease. J Immunol. Dec 15 2000;165(12):7323-9. [Medline].

  7. Hashimoto T, Takizawa H, Yukimura K, Ohta K. Vogt-Koyanagi-Harada disease associated with brainstem encephalitis. J Clin Neurosci. Apr 2009;16(4):593-5. [Medline].

  8. Levinson RD, Du Z, Luo L, Holland GN, Rao NA, Reed EF, et al. KIR and HLA gene combinations in Vogt-Koyanagi-Harada disease. Hum Immunol. Jun 2008;69(6):349-53. [Medline].

  9. 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. Sep 1990;97(9):1137-42. [Medline].

  10. Nussenblatt RB, Palestine AG, Chan CC. Cyclosporin A therapy in the treatment of intraocular inflammatory disease resistant to systemic corticosteroids and cytotoxic agents. Am J Ophthalmol. Sep 1983;96(3):275-82. [Medline].

  11. Bacsal K, Wen DS, Chee SP. Concomitant choroidal inflammation during anterior segment recurrence in Vogt-Koyanagi-Harada disease. Am J Ophthalmol. Mar 2008;145(3):480-486. [Medline].

  12. Errera MH, Fardeau C, Cohen D, et al. Effect of the duration of immunomodulatory therapy on the clinical features of recurrent episodes in Vogt--Koyanagi--Harada disease. Acta Ophthalmol. Jun 2011;89(4):e357-66. [Medline].

  13. Chee SP, Jap A, Cheung CM. The prognostic value of angiography in Vogt-Koyanagi-Harada disease. Am J Ophthalmol. Dec 2010;150(6):888-93. [Medline].

  14. Maruko I, Iida T, Sugano Y, et al. Subfoveal choroidal thickness after treatment of Vogt-Koyanagi-Harada disease. Retina. Mar 2011;31(3):510-7. [Medline].

  15. Bouchenaki N, Herbort CP. Indocyanine green angiography guided management of vogt-koyanagi-harada disease. J Ophthalmic Vis Res. Oct 2011;6(4):241-8. [Medline]. [Full Text].

  16. Ondrey FG, Moldestad E, Mastroianni MA, Pikus A, Sklare D, Vernon E, et al. Sensorineural hearing loss in Vogt-Koyanagi-Harada syndrome. Laryngoscope. Oct 2006;116(10):1873-6. [Medline].

  17. Cuchacovich M, Solanes F, Díaz G, et al. Comparison of the clinical efficacy of two different immunosuppressive regimens in patients with chronic vogt-koyanagi-harada disease. Ocul Immunol Inflamm. Jun 2010;18(3):200-7. [Medline].

  18. Read RW, Yu F, Accorinti M, Bodaghi B, Chee SP, Fardeau C. Evaluation of the effect on outcomes of the route of administration of corticosteroids in acute Vogt-Koyanagi-Harada disease. Am J Ophthalmol. Jul 2006;142(1):119-24. [Medline].

  19. Babel J. Syndrome de Vogt-Koyanagi. Schweiz Med Wochenscher. 1932;44:1136.

  20. Bouchenaki N, Herbort CP. The contribution of indocyanine green angiography to the appraisal and management of Vogt-Koyanagi-Harada disease. Ophthalmology. Jan 2001;108(1):54-64. [Medline].

  21. Bruno MG, McPherson SD Jr. Harada's disease. Am J Ophthalmol. 1949;32:513.

  22. Chan CC, Palestine AG, Nussenblatt RB, Roberge FG, Benezra D. Anti-retinal auto-antibodies in Vogt-Koyanagi-Harada syndrome, Behcet's disease, and sympathetic ophthalmia. Ophthalmology. Aug 1985;92(8):1025-8. [Medline].

  23. Cowper AR. Harada's disease and Vogt-Koyanagi syndrome: Uveoencephalitis. Arch Ophthalmol. 1951;45:367.

  24. Fine SB, Gilligan JH. The Vogt-Koyanagi-Harada syndrome. A variant of sympathetic ophthalmia. Report of two cases. Am J Ophthalmol. 1957;53:433.

  25. Forster DJ, Cano MR, Green RL, Rao NA. Echographic features of the Vogt-Koyanagi-Harada syndrome. Arch Ophthalmol. Oct 1990;108(10):1421-6. [Medline].

  26. Harada Y. Beitrag Zur klinischen Kenntnis von nichteitriger Choroiditis (Choroiditis diffusa acta). Acta Soc Ophthalmol Jpn. 1926;30:356.

  27. Ibanez HE, Grand MG, Meredith TA, Wippold FJ 2nd. Magnetic resonance imaging findings in Vogt-Koyanagi-Harada syndrome. Retina. 1994;14(2):164-8. [Medline].

  28. Islam SM, Numaga J, Matsuki K, Fujino Y, Maeda H, Masuda K. Influence of HLA-DRB1 gene variation on the clinical course of Vogt-Koyanagi-Harada disease. Invest Ophthalmol Vis Sci. Feb 1994;35(2):752-6. [Medline].

  29. Jabs DA, Rosenbaum JT, Foster CS, Holland GN, Jaffe GJ, Louie JS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. Oct 2000;130(4):492-513. [Medline].

  30. Koyanagi Y. Dysakusis, alopecia und poliosis bei schwerer Uveitis nicht traumatichen Ursprungs. Klin Monatsbl Augenheilkd. 1929;82:194.

  31. Lacerda RR. Sindrome de Vogt-Koyanagi-Harada: descricao de um caso especial em crianca de sete anos de idade. 1994;57:46-48.

  32. Lubin JR, Ni C, Albert DM. Clinicopathological study of the Vogt-Koyanagi-Harada syndrome. Int Ophthalmol Clin. 1982;22(3):141-56. [Medline].

  33. Maezawa N, Yano A, Taniguchi M, Kojima S. The role of cytotoxic T lymphocytes in the pathogenesis of Vogt-Koyanagi-Harada disease. Ophthalmologica. 1982;185(3):179-86. [Medline].

  34. Martinez JA, Lopez PF, Sternberg P Jr, Aaberg TM, Lambert HM, Capone A Jr, et al. Vogt-Koyanagi-Harada syndrome in patients with Cherokee Indian ancestry. Am J Ophthalmol. Nov 15 1992;114(5):615-20. [Medline].

  35. Momoeda S. [Lymphocyte transformation test in Vogt-Koyanagi-Harada syndrome (author's transl)]. Nippon Ganka Gakkai Zasshi. Aug 10 1976;80(8):491-6. [Medline].

  36. Morris WR, Schlaegel TF Jr. Viruslike inclusion bodies in subretinal fluid in uveoencephalitis. Am J Ophthalmol. 1964;58:940-945.

  37. Ohno S. Immunological aspects of Behçet's and Vogt-Koyanagi-Harada's diseases. Trans Ophthalmol Soc U K. Sep 1981;101 (Pt 3)(3):335-41. [Medline].

  38. Pattison EM. Uveomeningoencephalitic syndrome (Vogt-Koyanagi-Harada). Arch Neurol. 1965;12:197-205.

  39. Perry HD, Font RL. Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. Feb 1977;83(2):242-54. [Medline].

  40. Rao NA. Mechanisms of inflammatory response in sympathetic ophthalmia and VKH syndrome. Eye. 1997;11 (Pt 2):213-6. [Medline].

  41. Rao NA, Marak GE. Sympathetic ophthalmia simulating vogt-Koyanagi-Harada's disease: a clinico-pathologic study of four cases. Jpn J Ophthalmol. 1983;27(3):506-11. [Medline].

  42. Read RW. Vogt-Koyanagi-Harada disease. Ophthalmol Clin North Am. Sep 2002;15(3):333-41, vii. [Medline].

  43. 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. May 2001;131(5):647-52. [Medline].

  44. Reed H. The uveoencephalitic syndrome of Vogt-Koyanagi-Harada disease. Can Med Assoc J. 1958;79:451.

  45. Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical course, therapy, and long-term visual outcome. Arch Ophthalmol. May 1991;109(5):682-7. [Medline].

  46. Sagiura S. Vogt-Koyanagi-Harada disease. Jpn J Ophthalmol. 1978;22:9-35.

  47. Vogt A. Fruhzeitges Ergraunen der Zilien und Bemerkungen uber den sogenannten plotzlichen Eintritt dieser Veranderung. Klin Monatsbl Augenheilkd. 1906;44:228.

  48. Wang Y, Gaudio PA. Infliximab therapy for 2 patients with Vogt-Koyanagi-Harada syndrome. Ocul Immunol Inflamm. Jul-Aug 2008;16(4):167-71. [Medline].

  49. 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. Aug 2007;144(2):260-5. [Medline].

  50. Yuasa T, Murai Y, Hoki T, Mimura Y. [Lymphocyte transformation test and migration inhibition test of macrophages in Vogt-Koyanagi-Hirada's syndrome (author's transl)]. Nippon Ganka Gakkai Zasshi. Oct 1973;77(10):1652-7. [Medline].

  51. Yuge T. The relation between Vogt-Koyanagi syndrome and sympathetic ophthalmia. Report of a case of a case of Vogt-Koyanagi syndrome. Am J Ophthalmol. 1957;43:735.

 
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Bilateral multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye.
Fluorescein angiography of the left eye in a patient with Vogt-Koyanagi-Harada disease. Mid phase is shown on the left with multiple areas of hyperfluorescence at the level of the retinal pigment epithelium. Late phase of the same angiogram (right) reveals multiple placoid areas of hyperfluorescence at the level of the retinal pigment epithelium and pooling of dye in the areas of serous detachment.
Patient with progressive dysacusis and recent onset of visual loss. Fundus photo shows a large multifocal serous detachment of the right eye. B-scan ultrasonography reveals posterior choroidal thickening with an overlying retinal detachment.
Patient with progressive dysacusis and recent onset of visual loss is shown here following 6 weeks of systemic corticosteroid therapy. Diffuse depigmentation of the choroid with retinal pigment epithelium migration is seen. Residual retinal striae are present in the peripapillary region. B-scan ultrasonography shows resolution of retinal detachment and choroidal thickening.
 
 
 
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