Presentation
Patients often do not present to their ophthalmologist until this stage, when they experience acute ocular pain and red eyes associated with bilateral blurring of vision secondary to uveitis. [aao.org]
An 80-year-old man presented with poliosis that developed suddenly after having surgery for macular degeneration of his right eye about 10 years ago. [kyushu-u.pure.elsevier.com]
Patients are often typically middle-aged (20 to 50 years old) at presentation 7. [radiopaedia.org]
Case Report A 28-year-old Hispanic man presented with a 10-day history of red eyes and decreased vision bilaterally, with mild headaches. [ajnr.org]
Case Presentation An 18-year old woman with a history of astigmatism presented with the complaint of blurred vision in the right eye for two weeks. [omicsonline.org]
Entire Body System
- Fatigue
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Gastrointestinal
- Nausea
There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]
Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]
Initial onset of Vogt-Koyanagi-Harada syndrome is characterized by severe headache, deep pain in the eye, vertigo (feeling that the room is spinning), and nausea, followed in a few weeks by uveitis and vision loss. [healthcentral.com]
The prodromic stage is characterized by nonspecific symptoms such as fever, headaches, nausea and vertigo, and then by neurologic symptoms such as muscle weakness, hemiparesis, hemiplegia, dysarthria and orbital pain. [orpha.net]
Eyes
- Blurred Vision
A 57-year-old man with no family history of neurologic disease was healthy until age 40 years, when he gradually experienced bilateral eye pain and ``blurred'' vision. [n.neurology.org]
In the ophthalmologic stage, occurring a few days after the prodromic stage, patients complain of blurred vision, ocular pain, and photophobia or central scotoma (bilateral in 80% of cases). [orpha.net]
Case Presentation An 18-year old woman with a history of astigmatism presented with the complaint of blurred vision in the right eye for two weeks. [omicsonline.org]
The main symptom of the acute stage of VKH is blurred vision in both eyes [1]. Ophthalmological findings are panuveitis or posterior uveitis, with multifocal serous retinal detachments [2]. [dermnetnz.org]
Ears
- Tinnitus
There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]
The ocular symptoms were accompanied by tinnitus and marked dyspnea. Pulmonary consultation identified nonspecific pulmonary findings which were out of proportion to her dyspnea. [karger.com]
[…] multifocal exudative retinal or retinal pigment epithelial detachments, disc hyperemia or edema, or “sunset glow” fundus, which is a yellow-orange appearance of the fundus due to depigmentation of the RPE and choroid, 3) neurologic signs, including tinnitus [aao.org]
During the initial assessment by the Ophthalmologist, she complained about persistent tinnitus and headache, considered as unspecific by the Otorhinolaryngologist and Neurologist. [omicsonline.org]
Other features include the central nervous system and cutaneous origin, including deafness (sensorineural), headache, tinnitus, dysacousis, and meningism associated with cerebrospinal fluid pleocytosis. [accessanesthesiology.mhmedical.com]
- Dysacusis
In a retrospective review of 48 patients, Beniz et al. report the prevalence of headache (67%), tinnitus (17%), dysacusis (13%), vitiligo (10%), alopecia (13%), and poliosis (6%). [omicsonline.org]
Neurologic symptoms tend to occur early and include tinnitus, dysacusis (auditory agnosia), vertigo, headache, and meningismus. [msdmanuals.com]
Vogt in 1906 • Primary posterior uveitis +exudative RDs +CSF pleocytosis Einosuke Harada • B/l chronic iridocyclitis + skin & hair depigmentation Koyanagi • This constellation of findings was termed “uveitis with polosis, vitiligo, alopecia and dysacusis [de.slideshare.net]
Inner ear involvement Changes in the inner ear, such as dysacusis, hearing loss and vertigo, have been observed in 70 % of patients, especially during the prodromal stage. Tinnitus is present in 42 % [ 65 ]. [doi.org]
- Hearing Impairment
Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. [doi.org]
- Hearing Problem
Of the cases of VKH with hearing problems, 75 % returned to normality; however, although very rarely, VKH disease can cause profound hearing loss.11Immunosuppressive therapy The usefulness of IST has been widely demonstrated in patients with autoimmune [elsevier.es]
Skin
- Alopecia
[…] depigmented Hair), Hearing [..] 0 0 A syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING [..] [dictionary.university]
Vogt-Koyanagi syndrome - bilateral uveitis with iritis and glaucoma, premature graying of the hair, alopecia, vitiligo, and dysacusia. [medical-dictionary.thefreedictionary.com]
It is manifested with hearing disorders, panuveitis, alopecia, poliosis, vitiligo, neck stiffness, headaches, and focal neurologic signs ICD-9-CM Volume 2 Index entries containing back-references to 364.24 : [icd9data.com]
Alopecia-Poliosis-Uveitis-Vitiligo-Deafness-Cutaneous-Uveo-Oto Syndrome; Harada Syndrome; Harada Disease; Vogt-Koyanagi Syndrome; Yugé Syndrome; Oculocutaneous Syndrome; Uveocutaneous Syndrome; Uveitis-Vitiligo-Alopecia-Poliosis Syndrome; Uveomeningeal [accessanesthesiology.mhmedical.com]
- Cutaneous Manifestation
Given that the cutaneous manifestations of VKH disease are so characteristic, these could not have been missed even before the actual clinical entity of VKH was recognized in the early 20th century. [ncbi.nlm.nih.gov]
Vogt-Koyanagi-Harada syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. [radiopaedia.org]
Requires three of the following features: bilateral iridocyclitis, posterior uveitis (including serous retinal detachment or sunset glow fundus), central nervous system manifestations, and cutaneous manifestations. [accessanesthesiology.mhmedical.com]
- Piebaldism
The cutaneous symptoms may lead us to consider diagnostic possibilities such as Alezzandrini's syndrome, alopecia areata, vitiligo and piebaldism 10. [scielo.br]
Besides that, the cutaneous manifestations should exclude diagnosis such as Alezzandrini’s syndrome, alopecia areata, vitiligo and piebaldism. Other immunomediated sensorineural hearing loss should also be excluded such as Cogan syndrome. [doi.org]
Neurologic
- Meningism
Examination of the cerebrospinal fluid reveals a pattern consistent with meningitis, aseptic. [icd9data.com]
Examination of the cerebrospinal fluid reveals a pattern consistent with MENINGITIS, ASEPTIC. [mesh.kib.ki.se]
Historical note and terminology The subject of this review is a clinical syndrome in which pigmentary alterations in various tissues are associated with ocular and meningeal inflammation. [medlink.com]
- Headache
A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days. Best-corrected visual acuity was 20/20 in both his eyes. [ncbi.nlm.nih.gov]
It is manifested with hearing disorders, panuveitis, alopecia, poliosis, vitiligo, neck stiffness, headaches, and focal neurologic signs ICD-9-CM Volume 2 Index entries containing back-references to 364.24 : [icd9data.com]
The patient was given prednisolone acetate eye drops and oral prednisone, and his vision and headaches gradually improved during the next several weeks. By 6 months, the headaches did not recur and the visual acuity did not decline further. [ajnr.org]
[…] syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING DISORDERS, and meningeal signs (neck stiffness and headache [mesh.kib.ki.se]
Shortly after the onset of her symptoms, she experienced a severe headache localized to her temples. Although she had a history of frequent headaches, she describes the new headache to be unlike any prior episodes. [omicsonline.org]
- Vertigo
There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]
Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]
Initial onset of Vogt-Koyanagi-Harada syndrome is characterized by severe headache, deep pain in the eye, vertigo (feeling that the room is spinning), and nausea, followed in a few weeks by uveitis and vision loss. [healthcentral.com]
The prodromic stage is characterized by nonspecific symptoms such as fever, headaches, nausea and vertigo, and then by neurologic symptoms such as muscle weakness, hemiparesis, hemiplegia, dysarthria and orbital pain. [orpha.net]
- Neurologic Manifestation
In this update, the author cites work that shows that earlier initiation of either glucocorticoids or immunomodulatory measures significantly reduces late visual morbidity in patients with Vogt-Koyanagi-Harada syndrome and that the neurologic manifestations [medlink.com]
manifestation, 054078, 症状, ショウジョウ, symptom, 040039 [togodb.biosciencedbc.jp]
Incomplete VKH disease: similar eye disease as patients with complete VKH disease but do not have both neurologic and dermatological signs. Patients must have either the neurologic manifestations or dermatological signs. c. [rarediseases.org]
Patients with complete VKH disease must also have evidence of neurologic and auditory manifestations, as well as integumentary signs. However, the neurologic and auditory manifestations may resolve before an ophthalmic examination. [reference.medscape.com]
- Dizziness
Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (e.g. hair loss, poliosis, vitiligo). [radiopaedia.org]
Hearing disturbances (75%) and dizziness may also be present. [orpha.net]
The diagnosis of clinical deafness, tinnitus and dizziness should always consider Vogt-Koyanagi-Harada syndrome. [arquivosdeorl.org.br]
Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]
Workup
(See Presentation and Workup.) [6, 7, 8, 9, 10] Bilateral, multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye. [reference.medscape.com]
Exudative reti- TABLE 1 Diagnostic workup for Vogt-Koyanagi-Harada disease nal detachments in uveal effusion syndrome tend to be chronic and do not respond to corticosteroid treatment. [slideshare.net]
HLA Type
- HLA-DR4
The association of HLA-DR4/ HLA-DRB1*04 with VKH was reported in various ethnic populations. Our meta-analysis confirms these previous reports on the association of HLA-DR4/ HLA-DRB1*04 with VKH. [nature.com]
VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. [doi.org]
[…] found an association with HLA-DR4 and HLA-DR53, while HLA-DR1 and HLA-DR4 were reported in Hispanic patients living in southern California. [40] An association with HLA-DR4, HLA-DRw53, and HLA-DQw3 has been found in subjects of Native American ancestry [reference.medscape.com]
Treatment
Standard Therapies Treatment Standard treatment of Vogt-Koyanagi-Harada disease is the use of high-dose systemic steroid drugs initially and often followed by immunosuppressive therapy. [rarediseases.org]
This anti-TNF agent seemed to be an effective and well-tolerated treatment for these patients. [iovs.arvojournals.org]
With treatment, the visual outcome of VKH is generally good. [aao.org]
Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment. [doi.org]
Prognosis
Prognosis With early and aggressive treatment, the prognosis is usually favorable, but acute hearing and vision alterations may occur. [orpha.net]
Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment. [doi.org]
Prognosis The important action for a positive prognosis is an immediate start of therapy by taking high doses of anti-inflammatory medication in order to control inflammation (40). [arquivosdeorl.org.br]
Etiology
Diagnosis - Etiology en_US dc.subject.mesh Male en_US dc.subject.mesh Uveitis - Diagnosis - Etiology en_US dc.subject.mesh Uveomeningoencephalitic Syndrome - Complications - Drug Therapy en_US dc.title High-grade lymphoma after azathioprine treatment [hub.hku.hk]
Etiology Pathogenesis has been linked to immunological disorders targeting the melanocytes, and which involve T-cell-mediated cytotoxicity and apoptosis. However, the etiology is still not completely understood. [orpha.net]
Year introduced: 1991(1965) PubMed search builder options Subheadings: analysis anatomy and histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology ethnology etiology [ncbi.nlm.nih.gov]
Subject Adult; Evoked Potentials, Visual; Female; Humans; Pilocarpine/administration & dosage; Pupil/drug effects; Tonic Pupil/etiology; Uveomeningoencephalitic Syndrome/complications/diagnosis; Visual Acuity Abstract We report a 38-year-old woman with [collections.lib.utah.edu]
The etiology is unknown, but it may be an inflammatory autoimmune condition. Called also Harada syndrome. [medical-dictionary.thefreedictionary.com]
Epidemiology
1995 Jan;39(4):265-292) Year introduced: 1991(1965) PubMed search builder options Subheadings: analysis anatomy and histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology [ncbi.nlm.nih.gov]
Summary Epidemiology Annual incidence is estimated at 1/400,000. The condition predominantly affects young women and individuals with darker pigmentation (those of Asian, Hispanic, or Native American origin). [orpha.net]
Sciences Toxoplasmosis Medicine & Life Sciences Uveomeningoencephalitic Syndrome Medicine & Life Sciences Uveitis Medicine & Life Sciences Ocular Hypotension Medicine & Life Sciences Iris Medicine & Life Sciences Projects 2016 2018 3 Finished Zika virus epidemiology [pure.urosario.edu.co]
(See Etiology and Epidemiology.) [1, 2, 3, 4, 5] Independently, Vogt, Koyanagi, and Harada described several patients during a 20-year period with bilateral uveitis, exudative retinal detachments, neurologic abnormalities, and disorders of the integument [reference.medscape.com]
Pathophysiology
[…] syndrome was first reported in the 10th century by Ali Ibn Isa, an ophthalmologist in medieval Baghdad, who described the characteristic physical appearance of ocular inflammation with a distinct whitening of the hair, eyebrows, and eyelashes. 1 The pathophysiology [ajnr.org]
The underlying pathophysiology involves an immune-mediated attack of pigmented cells in the affected organ systems. Consequently, VKH syndrome is more common in individuals with darker skin pigmentation. [omicsonline.org]
[…] fibrosis; less frequently, posterior synechiae, neovascularization of the optic disc, arteriovenous anastomoses and choroidal neovascularization occur.4,6,7 All these complications severely compromise the visual prognosis.Etiology and pathogenesis The pathophysiology [elsevier.es]
It may also contribute to the understanding of the pathophysiology of chorioretinal inflammatory disordes [ 99 ]. [doi.org]
Prevention
[…] histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology ethnology etiology genetics history immunology metabolism microbiology pathology physiology physiopathology prevention [ncbi.nlm.nih.gov]
Treatment is important and can prevent serious complications. Make sure you report any new symptom to your doctor, and go to your follow-up visits as your doctor says. [webmd.com]
Others • Patients with inflammatory cell infiltration in the anterior chamber require topical corticosteroids and cycloplegics to reduce ciliary spasm and prevent posterior synechiae formation 55. [de.slideshare.net]
Early recognition and treatment can prevent progression to chronic convalescent and chronic recurrent stages of the disease [7]. [dermnetnz.org]