MCID: VGT001
MIFTS: 61

Vogt-Koyanagi-Harada Disease malady

Rare diseases, Neuronal diseases, Eye diseases, Immune diseases categories
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Summaries for Vogt-Koyanagi-Harada Disease

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NIH Rare Diseases:42 Vogt-koyanagi-harada syndrome is a condition that involves chronic inflammation of melanocytes, which are specialized cells that produce a pigment called melanin.  melanin is the substance that gives skin, hair, and eyes their color.  melanin is also found in the retina, where it plays a role in normal vision.  individuals with vogt-koyanagi-harada syndrome usually develop vision and hearing disturbances first, followed by signs of skin problems.  research suggests this condition is an autoimmune disease.  vogt-koyanagi-harada syndrome is more common in individuals with darker skin pigmentation including asian, middle eastern, hispanic, and native american populations. last updated: 3/14/2014

MalaCards based summary: Vogt-Koyanagi-Harada Disease, also known as uveomeningoencephalitic syndrome, is related to uveitis and vitiligo, and has symptoms including mild visual loss/impaired visual acuity, absent/decreased/thin eyebrows and absent/decreased lashes. An important gene associated with Vogt-Koyanagi-Harada Disease is PTPN22 (protein tyrosine phosphatase, non-receptor type 22 (lymphoid)), and among its related pathways are Hematopoietic cell lineage and IL12 signaling mediated by STAT4. The compounds gold sodium thiomalate and ranitidine have been mentioned in the context of this disorder. Affiliated tissues include eye, skin and retina, and related mouse phenotypes are renal/urinary system and digestive/alimentary.

Disease Ontology:8 A hypersensitivity reaction type ii disease that is caused by t helper cell mediated autoimmune attack of melanocytes resulting in inflammation of the inside of the eye, whitening of hair, skin pigment loss, and meningitis.

Aliases & Classifications for Vogt-Koyanagi-Harada Disease

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Sources:
8Disease Ontology, 10DISEASES, 44Novoseek, 48Orphanet, 62UMLS, 42NIH Rare Diseases, 27ICD9CM, 39NCIt, 57SNOMED-CT, 34MeSH, 26ICD10 via Orphanet
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Vogt-Koyanagi-Harada Disease, Aliases & Descriptions:

Name: Vogt-Koyanagi-Harada Disease 8 10 44 48
Uveomeningoencephalitic Syndrome 8 62
Vogt-Koyanagi-Harada Syndrome 42
Vogt-Koyanagi Syndrome 8
 
Uveomenigitic Syndrome 48
Harada's Disease 8
Vkh Syndrome 42


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Neuronal diseases, Eye diseases, Immune diseases


Characteristics (Orphanet epidemiological data):

48
vogt-koyanagi-harada disease:
Inheritance: Multigenic/multifactorial; Age of onset: Variable; Age of death: Normal


External Ids:

Disease Ontology8 DOID:12297
ICD9CM27 363.22, 364.24
NCIt39 C85218
MeSH34 D014607
ICD10 via Orphanet26 H20.8, H30.8

Related Diseases for Vogt-Koyanagi-Harada Disease

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Diseases related to Vogt-Koyanagi-Harada Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 114)
idRelated DiseaseScoreTop Affiliating Genes
1uveitis31.9IL2RA
2vitiligo31.5PTPN22, TYR
3hepatitis c31.3FAS, HLA-DRB1
4hepatitis31.2FASLG, FAS, HLA-DRB1
5type 1 diabetes mellitus30.9FASLG, HLA-DRB1
6psoriasis30.9IL2RA, HLA-DRB1, PTPN22
7diabetes mellitus30.9PTPN22, HLA-DRB1
8melanoma30.6HLA-DRB1, FASLG, FAS, TYR
9viral hepatitis30.6HLA-DRB1, FAS, FASLG
10leukemia30.0IL2RA, FASLG, FAS, HLA-DRB1, TYR
11graves' disease29.7HLA-DRB1, PTPN22, FAS, FASLG, TYR, IL2RA
12choroiditis11.1
13retinitis11.0
14sympathetic ophthalmia10.8
15headache10.8
16alopecia10.7
17behcet's disease10.7
18neuropathy10.7
19retinal detachment10.7
20aseptic meningitis10.6
21anterior uveitis10.6
22cataract10.6
23posterior scleritis10.6
24meningoencephalitis10.6
25scleritis10.6
26acute posterior multifocal placoid pigment epitheliopathy10.6
27ankylosing spondylitis10.4
28intraocular lymphoma10.4
29keratopathy10.4
30keratoconjunctivitis sicca10.4
31pars planitis10.4
32alopecia areata10.4
33keratoconjunctivitis10.4
34panuveitis10.4
35spondylitis10.4
36viral meningitis10.4
37halo nevi10.4
38hyperacusis10.4
39cutaneous malignant melanoma10.4
40idiopathic anterior uveitis10.4
41leprosy10.4HLA-DRB1
42acute lymphoblastic leukemia10.4
43hodgkin's lymphoma10.4
44ischemic optic neuropathy10.4
45polycystic ovary syndrome10.4
46relapsing polychondritis10.4
47macular holes10.4
48myelitis10.4
49sensorineural hearing loss10.4
50glucose intolerance10.4

Graphical network of the top 20 diseases related to Vogt-Koyanagi-Harada Disease:



Diseases related to vogt-koyanagi-harada disease

Symptoms for Vogt-Koyanagi-Harada Disease

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Symptoms:

48 (show all 13)
  • mild visual loss/impaired visual acuity
  • absent/decreased/thin eyebrows
  • absent/decreased lashes
  • sensorineural deafness/hearing loss
  • irregular/patchy skin hypopigmentation
  • hypotrichosis/atrichia/atrichiasis/scalp hairlessness
  • premature greying of hair
  • intellectual deficit/mental/psychomotor retardation/learning disability
  • glaucoma
  • cataract/lens opacification
  • retinal detachment
  • visual loss/blindness/amblyopia
  • short stature/dwarfism/nanism

HPO human phenotypes related to Vogt-Koyanagi-Harada Disease:

(show all 13)
id Description Frequency HPO Source Accession
1 sensorineural hearing impairment hallmark (90%) HP:0000407
2 abnormality of the eyelashes hallmark (90%) HP:0000499
3 visual impairment hallmark (90%) HP:0000505
4 hypopigmented skin patches hallmark (90%) HP:0001053
5 premature graying of hair hallmark (90%) HP:0002216
6 abnormal hair quantity hallmark (90%) HP:0011362
7 cognitive impairment hallmark (90%) HP:0100543
8 aplasia/hypoplasia of the eyebrow hallmark (90%) HP:0100840
9 glaucoma typical (50%) HP:0000501
10 visual impairment typical (50%) HP:0000505
11 cataract typical (50%) HP:0000518
12 retinal detachment typical (50%) HP:0000541
13 short stature typical (50%) HP:0004322

Drugs & Therapeutics for Vogt-Koyanagi-Harada Disease

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Drug clinical trials:

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Search NIH Clinical Center for Vogt-Koyanagi-Harada Disease

Genetic Tests for Vogt-Koyanagi-Harada Disease

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Anatomical Context for Vogt-Koyanagi-Harada Disease

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MalaCards organs/tissues related to Vogt-Koyanagi-Harada Disease:

32
Eye, Skin, Retina, T cells, Spinal cord, Liver, Ovary

Animal Models for Vogt-Koyanagi-Harada Disease or affiliated genes

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MGI Mouse Phenotypes related to Vogt-Koyanagi-Harada Disease:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053678.3FASLG, FAS, PTPN22, TYR
2MP:00053818.2PTPN22, FAS, FASLG, IL2RA
3MP:00053797.9TYR, PTPN22, FAS, FASLG
4MP:00107687.8IL2RA, FASLG, FAS, PTPN22, TYR
5MP:00053877.7TYR, PTPN22, FAS, FASLG, IL2RA

Publications for Vogt-Koyanagi-Harada Disease

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Articles related to Vogt-Koyanagi-Harada Disease:

(show top 50)    (show all 285)
idTitleAuthorsYear
1
Association of HLA-DR4/HLA-DRB1*04 with Vogt-Koyanagi-Harada disease: A Systematic Review and Meta-analysis. (25382027)
2014
2
Decreased Interleukin-37 Expression in Vogt-Koyanagi-Harada Disease and Upregulation Following Immunosuppressive Treatment. (25343528)
2014
3
Influence of molecular genetics in Vogt-Koyanagi-Harada disease. (25097674)
2014
4
Choroidal atrophy and loss of choriocapillaris in convalescent stage of Vogt-Koyanagi-Harada disease: in vivo documentation. (24655594)
2014
5
Vogt-koyanagi-harada disease in an adolescent boy. (23869531)
2014
6
Vogt-Koyanagi-Harada disease in First Nations and MActis of Northern Alberta. (23769774)
2013
7
Retinal pigment epithelium folds as a diagnostic finding of Vogt-Koyanagi-Harada disease. (23149670)
2013
8
Indocyanine green angiography-guided management of Vogt-Koyanagi-Harada disease: differentiation between choroidal scars and active lesions. (23277207)
2013
9
Vogt-Koyanagi-Harada disease masquerading as a lymphoproliferative process of the uvea. (23913248)
2013
10
Vogt-Koyanagi-Harada disease: a forgotten entity in the Canadian uveitis landscape? (23769771)
2013
11
Vogt-Koyanagi-Harada disease in Thailand. (23163754)
2012
12
Probable Vogt-Koyanagi-Harada disease with initial unilateral ocular manifestation in a hepatitis C carrier. (22588777)
2012
13
Decreased IL-27 expression in association with an increased Th17 response in Vogt-Koyanagi-Harada disease. (22669715)
2012
14
Imaging in the diagnosis and management of Vogt-Koyanagi-Harada disease. (22954938)
2012
15
Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset. (22452682)
2011
16
Intravitreal triamcinolone acetonide for rebound phenomenon after high-dose intravenous steroid treatment in Vogt-Koyanagi-Harada disease. (22125406)
2011
17
Intravitreal bevacizumab injection for persistent serous retinal detachment associated with Vogt-Koyanagi-Harada disease. (20689964)
2011
18
Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease. (19151926)
2010
19
Blue light and near-infrared fundus autofluorescence in acute Vogt-Koyanagi-Harada disease. (19965835)
2010
20
Morphologic changes in the anterior segment in patients with initial-onset or recurrent Vogt-Koyanagi-Harada disease. (20662662)
2010
21
The prognostic value of angiography in Vogt-Koyanagi-Harada disease. (20933219)
2010
22
Indocyanine green angiography findings in initial acute pretreatment Vogt-Koyanagi-Harada disease in Japanese patients. (21052896)
2010
23
Frequency of distinguishing clinical features in Vogt-Koyanagi-Harada disease. (20036008)
2010
24
Vogt-Koyanagi-Harada disease: diagnosis and treatments update. (20829689)
2010
25
Vogt-Koyanagi-Harada disease and vitiligo: where does the illness begin? (18174263)
2008
26
Elevated choroidal blood flow velocity during systemic corticosteroid therapy in Vogt-Koyanagi-Harada disease. (19016661)
2008
27
Thunderclap headache as initial manifestation of Vogt-Koyanagi-Harada disease. (17868357)
2008
28
Characteristics of Vogt-Koyanagi-Harada disease in a French cohort: ethnicity, systemic manifestations, and HLA genotype data. (18379934)
2008
29
Vitreous surgery for macular hole in patients with Vogt-Koyanagi-Harada disease. (19278482)
2008
30
Concomitant choroidal inflammation during anterior segment recurrence in Vogt-Koyanagi-Harada disease. (18191100)
2008
31
Vogt-Koyanagi-Harada disease: inquiry into the genesis of a disease name in the historical context of Switzerland and Japan. (17468832)
2007
32
Cross-reaction between tyrosinase peptides and cytomegalovirus antigen by T cells from patients with Vogt-Koyanagi-Harada disease. (17253112)
2007
33
The spectrum of Vogt-Koyanagi-Harada disease in Turkey: VKH in Turkey. (16957877)
2007
34
IL-23 promotes CD4+ T cells to produce IL-17 in Vogt-Koyanagi-Harada disease. (17335887)
2007
35
Vogt-koyanagi-harada disease with onset in elderly patients aged 68 to 89 years. (17295144)
2007
36
Pathology of Vogt-Koyanagi-Harada disease. (17435969)
2007
37
Vogt-Koyanagi-Harada disease. (16770158)
2006
38
Vogt-Koyanagi-Harada disease in patients with chronic hepatitis C. (16310490)
2005
39
Early manifestation of vogt-koyanagi-harada disease as unilateral posterior scleritis. (12457922)
2002
40
Pathogenesis of Vogt-Koyanagi-Harada disease. (12189608)
2002
41
Sunset glow fundus in Vogt-Koyanagi-Harada disease with or without chronic ocular inflammation. (12397437)
2002
42
Complications and prognostic factors in Vogt-Koyanagi-Harada disease. (11336934)
2001
43
Tyrosinase family proteins are antigens specific to Vogt-Koyanagi-Harada disease. (11120868)
2000
44
HLA-DRB1*0405 is the predominant allele in Brazilian patients with Vogt-Koyanagi-Harada disease. (9548078)
1998
45
Tyrosinase epitope recognized by an HLA-DR-restricted T-cell line from a Vogt-Koyanagi-Harada disease patient. (9510558)
1998
46
Vogt Koyanagi Harada disease. (2098028)
1990
47
Choroidal neovascularization in long-standing case of Vogt-Koyanagi-Harada disease. (6190031)
1983
48
Vitiligo with an inflammatory erythema in Vogt-Koyanagi-Harada disease: demonstration of filamentous masses and amyloid deposits. (7119218)
1982
49
Two cases of Vogt-Koyanagi-Harada disease. (13691273)
1960
50
Vogt-Koyanagi-Harada disease; treatment with prednisone. (13410236)
1957

Variations for Vogt-Koyanagi-Harada Disease

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Expression for genes affiliated with Vogt-Koyanagi-Harada Disease

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Expression patterns in normal tissues for genes affiliated with Vogt-Koyanagi-Harada Disease

Search GEO for disease gene expression data for Vogt-Koyanagi-Harada Disease.

Pathways for genes affiliated with Vogt-Koyanagi-Harada Disease

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Pathways related to Vogt-Koyanagi-Harada Disease according to GeneCards/GeneDecks:

(show all 20)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5HLA-DRB1, IL2RA
29.5IL2RA, HLA-DRB1
3
Show member pathways
Immune response IL 22 signaling pathway60
G protein signaling N RAS regulation pathway60
9.5HLA-DRB1, IL2RA
4
Show member pathways
Ras signaling in the CD4+ TCR pathway37
9.4IL2RA, FASLG
5
Show member pathways
IL2 signaling events mediated by STAT537
9.4FASLG, IL2RA
6
Show member pathways
Calcium signaling in the CD4+ TCR pathway37
9.4FASLG, IL2RA
7
Show member pathways
FAS pathway and Stress induction of HSP regulation37
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis37
9.0FAS, FASLG
8
Show member pathways
Transcription NF kB signaling pathway60
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response60
9.0FASLG, FAS
9
Show member pathways
TRAIL signaling pathway37
9.0FAS, FASLG
10
Show member pathways
Apoptosis and survival Apoptotic TNF family pathways60
9.0FAS, FASLG
119.0FAS, FASLG
129.0FAS, FASLG
13
Show member pathways
9.0FAS, FASLG
14
Show member pathways
Immune response IL 12 induced IFN gamma production60
8.9IL2RA, FASLG, HLA-DRB1
15
Show member pathways
8.6IL2RA, FASLG, FAS
16
Show member pathways
8.6IL2RA, FASLG, FAS
17
Show member pathways
Immune response CD16 signaling in NK cells60
8.5FAS, FASLG, KIR2DS3
188.2IL2RA, FASLG, FAS, PTPN22
19
Show member pathways
8.1FAS, HLA-DRB1, IL2RA, FASLG
20
Show member pathways
7.5IL2RA, KIR2DS3, HLA-DRB1, FASLG, FAS

Compounds for genes affiliated with Vogt-Koyanagi-Harada Disease

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Compounds related to Vogt-Koyanagi-Harada Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 76)
idCompoundScoreTop Affiliating Genes
1gold sodium thiomalate449.9IL2RA, HLA-DRB1
2ranitidine44 28 24 1112.9IL2RA, TYR
3selenite449.8IL2RA, TYR
4c-d4t449.8FASLG, IL2RA
5polysulfone449.6IL2RA, FASLG
6chitosan449.6FAS, TYR
7(glc)4449.4FAS, FASLG
8atpo28 4410.4FASLG, FAS
9sodium arsenite449.4FASLG, IL2RA
10nitrate449.4FASLG, IL2RA
11teniposide44 1110.4FAS, FASLG
12okt3449.3IL2RA, FAS
13methotrexate50 44 1111.2FASLG, HLA-DRB1, IL2RA
14concanamycin a44 6110.1FASLG, FAS, TYR
15c2ceramide449.0FAS, TYR, FASLG
162-chlorodeoxyadenosine449.0FAS, FASLG, IL2RA
17resveratrol44 61 24 1112.0TYR, FASLG, FAS
18vitamin d449.0FAS, TYR, IL2RA
19ivig449.0FASLG, FAS, IL2RA
20ifn-alpha449.0IL2RA, FASLG, FAS
21anisomycin44 1110.0FAS, IL2RA, FASLG
22agar449.0FASLG, TYR, FAS
23ionomycin448.9FAS, IL2RA, FASLG
243-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide448.9FAS, FASLG
255fluorouracil448.9IL2RA, FASLG, FAS
26rantes448.9FASLG, FAS, IL2RA
27sb 20358044 619.9FAS, FASLG, TYR
28epinephrine44 24 1110.9FAS, IL2RA, FASLG
29etoposide44 50 61 1111.9IL2RA, FASLG, FAS
30n acetylcysteine448.9IL2RA, FAS, FASLG
31dutp448.7FAS, FASLG
32rapamycin448.7FASLG, FAS, IL2RA
33ly294002448.7TYR, FASLG, FAS
34ascorbic acid44 249.6FASLG, TYR, IL2RA, FAS
35indomethacin44 28 61 1111.6FASLG, IL2RA, FAS, TYR
36polysaccharide448.6FASLG, TYR, FAS, IL2RA
37paclitaxel44 50 1110.6TYR, FASLG, IL2RA, FAS
38doxorubicin44 50 1110.6IL2RA, FASLG, FAS, TYR
39paraffin448.6FAS, TYR, FASLG, IL2RA
40genistein44 28 61 2 24 1113.6TYR, IL2RA, FASLG, FAS
41cisplatin44 50 61 1111.6IL2RA, TYR, FASLG, FAS
42aspartate448.6IL2RA, FASLG, FAS, TYR
43h2o2448.6IL2RA, FASLG, FAS, TYR
44testosterone44 61 24 1111.5FAS, TYR, FASLG, IL2RA
45dexamethasone44 50 28 1111.5IL2RA, TYR, FASLG, FAS
46glutamate448.5PTPN22, TYR, HLA-DRB1, IL2RA, FASLG
47vegf448.5FAS, TYR, IL2RA, FASLG
48cysteine448.4FASLG, IL2RA, TYR, FAS
49arginine448.3FAS, IL2RA, HLA-DRB1, TYR, PTPN22
50tyrosine447.7FAS, PTPN22, HLA-DRB1, TYR, IL2RA, FASLG

GO Terms for genes affiliated with Vogt-Koyanagi-Harada Disease

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Cellular components related to Vogt-Koyanagi-Harada Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1perinuclear region of cytoplasmGO:0484719.1FASLG, PTPN22, TYR
2external side of plasma membraneGO:0098977.8IL2RA, FASLG, FAS, HLA-DRB1

Biological processes related to Vogt-Koyanagi-Harada Disease according to GeneCards/GeneDecks:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1inflammatory response to antigenic stimulusGO:0024379.7IL2RA, HLA-DRB1
2negative regulation of T cell proliferationGO:0421309.6IL2RA, HLA-DRB1
3activation-induced cell death of T cellsGO:0069249.4IL2RA, FAS
4inflammatory cell apoptotic processGO:0069259.3FASLG, FAS
5necroptotic signaling pathwayGO:0975279.3FASLG, FAS
6regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:20012399.2FAS, FASLG
7extrinsic apoptotic signaling pathwayGO:0971919.1FAS, FASLG
8extrinsic apoptotic signaling pathway via death domain receptorsGO:0086259.1FAS, FASLG
9apoptotic signaling pathwayGO:0971909.0FASLG, FAS
10activation of cysteine-type endopeptidase activity involved in apoptotic processGO:0069198.9FAS, FASLG
11immune responseGO:0069558.9IL2RA, FASLG, HLA-DRB1
12apoptotic processGO:0069158.3IL2RA, FASLG, FAS

Molecular functions related to Vogt-Koyanagi-Harada Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1kinase bindingGO:0199009.3FAS, PTPN22

Products for genes affiliated with Vogt-Koyanagi-Harada Disease

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Sources for Vogt-Koyanagi-Harada Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet