Vogt-Koyanagi-Harada syndrome is a multisystem disorder characterised by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations.
Vogt-Koyanagi-Harada usually affected those of Asian, Middle Eastern, Asian Indian, Native American or Hispanic ethnicity 5. Patients are often typically middle-aged (20 to 50 years old) at presentation 7.
Patients with Vogt-Koyanagi-Harada disease usually initially present to an ophthalmologist for ocular problems, including sudden loss of vision, ocular pain and photophobia. Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (e.g. hair loss, poliosis, vitiligo).
It is considered an inflammatory syndrome occurring probably as a result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes.
B-mode ocular ultrasound features of Vogt-Koyanagi-Harada syndrome are:
- low to medium reflective thickening of the posterior choroid
- serous retinal detachments
- mild thickening of the sclera and/or episclera adjacent to areas of choroidal thickening
- vitreous opacities
- subretinal septations may be seen
MR imaging may detect early CNS involvement by Vogt-Koyanagi-Harada disease before the onset of neurologic symptoms which include:
- typical bilaterality of ocular findings
- scattered periventricular white matter lesions on T2-weighted imaging/FLAIR, and pachymeningeal enhancement.
- bilateral contrast enhancement of the choroid is seen along diffuse choroidal thickening with scleral sparing and retinal detachment
- 5. Read RW. Vogt-Koyanagi-Harada disease. Ophthalmol Clin North Am. 2002;15 (3): 333-41, vii. Pubmed citation
- 3. Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol. 1995;39 (4): 265-92. Pubmed citation
- 4. Fang W, Yang P. Vogt-koyanagi-harada syndrome. Curr. Eye Res. 2008;33 (7): 517-23. doi:10.1080/02713680802233968 - Pubmed citation
- 1. Ibanez HE, Grand MG, Meredith TA et-al. Magnetic resonance imaging findings in Vogt-Koyanagi-Harada syndrome. Retina (Philadelphia, Pa.). 1994;14 (2): 164-8. Pubmed citation
- 2. Gilbert JA, Pollack ES, Pollack CV. Vogt-Koyanagi-Harada syndrome: case report and review. J Emerg Med. 1995;12 (5): 615-9. Pubmed citation
- 6. Lohman BD, Gustafson CA, McKinney AM et-al. MR imaging of Vogt-Koyanagi-Harada syndrome with leptomeningeal enhancement. AJNR Am J Neuroradiol. 2011;32 (9): E169-71. doi:10.3174/ajnr.A2279 - Pubmed citation
- 7. Damico FM, Kiss S, Young LH. Vogt-Koyanagi-Harada disease. Semin Ophthalmol. 2005;20 (3): 183-90. doi:10.1080/08820530500232126 - Pubmed citation
- 8. Agrawal RV, Murthy S, Sangwan V et-al. Current approach in diagnosis and management of anterior uveitis. Indian J Ophthalmol. 2010;58 (1): 11-9. doi:10.4103/0301-4738.58468 - Free text at pubmed - Pubmed citation