MCID: VGT001
MIFTS: 53

Vogt-Koyanagi-Harada Disease malady

Rare diseases, Neuronal diseases, Eye diseases categories

Summaries for Vogt-Koyanagi-Harada Disease

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NIH Rare Diseases:41 Vogt-koyanagi-harada disease (vkh disease) 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. people with vkh disease usually develop vision and hearing disturbances first, followed by signs of skin problems. the most common symptoms include headaches, panuveitis, vitiligo, hair loss (alopecia), and inner ear disturbances. neurological symptoms may also occur. the exact cause of vkh disease is not well understood, but research suggests it is an autoimmune disease. it is more common in people with darker skin pigmentation including asian, middle eastern, hispanic, and native american populations. last updated: 1/8/2015

MalaCards based summary: Vogt-Koyanagi-Harada Disease, also known as uveomeningoencephalitic syndrome, is related to uveitis and hepatitis c, and has symptoms including sensorineural hearing impairment, abnormality of the eyelashes and visual impairment. 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:9 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:
9Disease Ontology, 41NIH Rare Diseases, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 55SNOMED-CT, 27ICD9CM, 33MeSH, 38NCIt, 26ICD10 via Orphanet
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Vogt-Koyanagi-Harada Disease, Aliases & Descriptions:

Name: Vogt-Koyanagi-Harada Disease 9 41 11 43 47
Uveomeningoencephalitic Syndrome 9 60
Uveomenigitic Syndrome 41 47
Vogt-Koyanagi-Harada Syndrome 41
 
Vogt-Koyanagi Syndrome 9
Harada's Disease 9
Vkh Syndrome 41
Vkh Disease 41


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
vogt-koyanagi-harada disease:
Inheritance: Multigenic/multifactorial; Age of onset: All ages; Age of death: normal life expectancy


External Ids:

Disease Ontology9 DOID:12297
ICD9CM27 363.22, 364.24
MeSH33 D014607
NCIt38 C85218
Orphanet47 3437
ICD10 via Orphanet26 H20.8, H30.8

Related Diseases for Vogt-Koyanagi-Harada Disease

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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:

 47 (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:

Search ClinicalTrials for Vogt-Koyanagi-Harada Disease

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:

31
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:

35
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 294)
idTitleAuthorsYear
1
Analysis of choroidal folds in acute Vogt-Koyanagi-Harada disease using high-penetration optical coherence tomography. (25663475)
2015
2
Posterior subtenon triamcinolone acetonide injection as a primary treatment in eyes with acute Vogt-Koyanagi-Harada disease. (25792626)
2015
3
Association of HLA-DR4/HLA-DRB1*04 with Vogt-Koyanagi-Harada disease: A Systematic Review and Meta-analysis. (25382027)
2014
4
Decreased Interleukin-37 Expression in Vogt-Koyanagi-Harada Disease and Upregulation Following Immunosuppressive Treatment. (25343528)
2014
5
Influence of molecular genetics in Vogt-Koyanagi-Harada disease. (25097674)
2014
6
Cluster-like headache as presenting feature of Vogt-Koyanagi-Harada disease. (25591688)
2014
7
Choroidal atrophy and loss of choriocapillaris in convalescent stage of Vogt-Koyanagi-Harada disease: in vivo documentation. (24655594)
2014
8
Vogt-koyanagi-harada disease in an adolescent boy. (23869531)
2014
9
Vogt-Koyanagi-Harada disease in First Nations and MActis of Northern Alberta. (23769774)
2013
10
Retinal pigment epithelium folds as a diagnostic finding of Vogt-Koyanagi-Harada disease. (23149670)
2013
11
Indocyanine green angiography-guided management of Vogt-Koyanagi-Harada disease: differentiation between choroidal scars and active lesions. (23277207)
2013
12
Vogt-Koyanagi-Harada disease masquerading as a lymphoproliferative process of the uvea. (23913248)
2013
13
Vogt-Koyanagi-Harada disease: a forgotten entity in the Canadian uveitis landscape? (23769771)
2013
14
Vogt-Koyanagi-Harada disease in Thailand. (23163754)
2012
15
Probable Vogt-Koyanagi-Harada disease with initial unilateral ocular manifestation in a hepatitis C carrier. (22588777)
2012
16
Decreased IL-27 expression in association with an increased Th17 response in Vogt-Koyanagi-Harada disease. (22669715)
2012
17
Imaging in the diagnosis and management of Vogt-Koyanagi-Harada disease. (22954938)
2012
18
Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset. (22452682)
2011
19
Intravitreal triamcinolone acetonide for rebound phenomenon after high-dose intravenous steroid treatment in Vogt-Koyanagi-Harada disease. (22125406)
2011
20
Intravitreal bevacizumab injection for persistent serous retinal detachment associated with Vogt-Koyanagi-Harada disease. (20689964)
2011
21
Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease. (19151926)
2010
22
Blue light and near-infrared fundus autofluorescence in acute Vogt-Koyanagi-Harada disease. (19965835)
2010
23
Morphologic changes in the anterior segment in patients with initial-onset or recurrent Vogt-Koyanagi-Harada disease. (20662662)
2010
24
The prognostic value of angiography in Vogt-Koyanagi-Harada disease. (20933219)
2010
25
Indocyanine green angiography findings in initial acute pretreatment Vogt-Koyanagi-Harada disease in Japanese patients. (21052896)
2010
26
Frequency of distinguishing clinical features in Vogt-Koyanagi-Harada disease. (20036008)
2010
27
Vogt-Koyanagi-Harada disease and vitiligo: where does the illness begin? (18174263)
2008
28
Elevated choroidal blood flow velocity during systemic corticosteroid therapy in Vogt-Koyanagi-Harada disease. (19016661)
2008
29
Thunderclap headache as initial manifestation of Vogt-Koyanagi-Harada disease. (17868357)
2008
30
Characteristics of Vogt-Koyanagi-Harada disease in a French cohort: ethnicity, systemic manifestations, and HLA genotype data. (18379934)
2008
31
Vitreous surgery for macular hole in patients with Vogt-Koyanagi-Harada disease. (19278482)
2008
32
Concomitant choroidal inflammation during anterior segment recurrence in Vogt-Koyanagi-Harada disease. (18191100)
2008
33
Vogt-Koyanagi-Harada disease: inquiry into the genesis of a disease name in the historical context of Switzerland and Japan. (17468832)
2007
34
Cross-reaction between tyrosinase peptides and cytomegalovirus antigen by T cells from patients with Vogt-Koyanagi-Harada disease. (17253112)
2007
35
The spectrum of Vogt-Koyanagi-Harada disease in Turkey: VKH in Turkey. (16957877)
2007
36
IL-23 promotes CD4+ T cells to produce IL-17 in Vogt-Koyanagi-Harada disease. (17335887)
2007
37
Vogt-koyanagi-harada disease with onset in elderly patients aged 68 to 89 years. (17295144)
2007
38
Pathology of Vogt-Koyanagi-Harada disease. (17435969)
2007
39
Vogt-Koyanagi-Harada disease. (16770158)
2006
40
Vogt-Koyanagi-Harada disease in patients with chronic hepatitis C. (16310490)
2005
41
Early manifestation of vogt-koyanagi-harada disease as unilateral posterior scleritis. (12457922)
2002
42
Pathogenesis of Vogt-Koyanagi-Harada disease. (12189608)
2002
43
Sunset glow fundus in Vogt-Koyanagi-Harada disease with or without chronic ocular inflammation. (12397437)
2002
44
Complications and prognostic factors in Vogt-Koyanagi-Harada disease. (11336934)
2001
45
Tyrosinase family proteins are antigens specific to Vogt-Koyanagi-Harada disease. (11120868)
2000
46
HLA-DRB1*0405 is the predominant allele in Brazilian patients with Vogt-Koyanagi-Harada disease. (9548078)
1998
47
Tyrosinase epitope recognized by an HLA-DR-restricted T-cell line from a Vogt-Koyanagi-Harada disease patient. (9510558)
1998
48
Choroidal neovascularization in long-standing case of Vogt-Koyanagi-Harada disease. (6190031)
1983
49
Vitiligo with an inflammatory erythema in Vogt-Koyanagi-Harada disease: demonstration of filamentous masses and amyloid deposits. (7119218)
1982
50
Two cases of Vogt-Koyanagi-Harada disease. (13691273)
1960

Variations for Vogt-Koyanagi-Harada Disease

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Expression for genes affiliated with Vogt-Koyanagi-Harada Disease

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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 Suite gene sharing:

(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 pathway58
G protein signaling N RAS regulation pathway58
9.5HLA-DRB1, IL2RA
4
Show member pathways
Ras signaling in the CD4+ TCR pathway36
9.4IL2RA, FASLG
5
Show member pathways
IL2 signaling events mediated by STAT536
9.4FASLG, IL2RA
6
Show member pathways
Calcium signaling in the CD4+ TCR pathway36
9.4FASLG, IL2RA
7
Show member pathways
FAS pathway and Stress induction of HSP regulation36
Apoptosis and survival FAS signaling cascades58
Caspase cascade in apoptosis36
9.0FAS, FASLG
8
Show member pathways
Transcription NF kB signaling pathway58
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response58
9.0FASLG, FAS
9
Show member pathways
TRAIL signaling pathway36
9.0FAS, FASLG
10
Show member pathways
Apoptosis and survival Apoptotic TNF family pathways58
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 production58
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 cells58
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 Suite gene sharing:

(show top 50)    (show all 76)
idCompoundScoreTop Affiliating Genes
1gold sodium thiomalate439.9IL2RA, HLA-DRB1
2ranitidine43 28 24 1212.9IL2RA, TYR
3selenite439.8IL2RA, TYR
4c-d4t439.8FASLG, IL2RA
5polysulfone439.6IL2RA, FASLG
6chitosan439.6FAS, TYR
7(glc)4439.4FAS, FASLG
8atpo28 4310.4FASLG, FAS
9sodium arsenite439.4FASLG, IL2RA
10nitrate439.4FASLG, IL2RA
11teniposide43 1210.4FAS, FASLG
12okt3439.3IL2RA, FAS
13methotrexate49 43 1211.2FASLG, HLA-DRB1, IL2RA
14concanamycin a43 5910.1FASLG, FAS, TYR
15c2ceramide439.0FAS, TYR, FASLG
162-chlorodeoxyadenosine439.0FAS, FASLG, IL2RA
17resveratrol43 59 24 1212.0TYR, FASLG, FAS
18vitamin d439.0FAS, TYR, IL2RA
19ivig439.0FASLG, FAS, IL2RA
20ifn-alpha439.0IL2RA, FASLG, FAS
21anisomycin43 1210.0FAS, IL2RA, FASLG
22agar439.0FASLG, TYR, FAS
23ionomycin438.9FAS, IL2RA, FASLG
243-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide438.9FAS, FASLG
255fluorouracil438.9IL2RA, FASLG, FAS
26rantes438.9FASLG, FAS, IL2RA
27sb 20358043 599.9FAS, FASLG, TYR
28epinephrine43 24 1210.9FAS, IL2RA, FASLG
29etoposide43 49 59 1211.9IL2RA, FASLG, FAS
30n acetylcysteine438.9IL2RA, FAS, FASLG
31dutp438.7FAS, FASLG
32rapamycin438.7FASLG, FAS, IL2RA
33ly294002438.7TYR, FASLG, FAS
34ascorbic acid43 249.6FASLG, TYR, IL2RA, FAS
35indomethacin43 28 59 1211.6FASLG, IL2RA, FAS, TYR
36polysaccharide438.6FASLG, TYR, FAS, IL2RA
37paclitaxel43 49 1210.6TYR, FASLG, IL2RA, FAS
38doxorubicin43 49 1210.6IL2RA, FASLG, FAS, TYR
39paraffin438.6FAS, TYR, FASLG, IL2RA
40genistein43 28 59 2 24 1213.6TYR, IL2RA, FASLG, FAS
41cisplatin43 49 59 1211.6IL2RA, TYR, FASLG, FAS
42aspartate438.6IL2RA, FASLG, FAS, TYR
43h2o2438.6IL2RA, FASLG, FAS, TYR
44testosterone43 59 24 1211.5FAS, TYR, FASLG, IL2RA
45dexamethasone43 49 28 1211.5IL2RA, TYR, FASLG, FAS
46glutamate438.5PTPN22, TYR, HLA-DRB1, IL2RA, FASLG
47vegf438.5FAS, TYR, IL2RA, FASLG
48cysteine438.4FASLG, IL2RA, TYR, FAS
49arginine438.3FAS, IL2RA, HLA-DRB1, TYR, PTPN22
50tyrosine437.7FAS, 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 Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1perinuclear region of cytoplasmGO:00484719.1FASLG, PTPN22, TYR
2external side of plasma membraneGO:00098977.8IL2RA, FASLG, FAS, HLA-DRB1

Biological processes related to Vogt-Koyanagi-Harada Disease according to GeneCards Suite gene sharing:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1inflammatory response to antigenic stimulusGO:00024379.7IL2RA, HLA-DRB1
2negative regulation of T cell proliferationGO:00421309.6IL2RA, HLA-DRB1
3activation-induced cell death of T cellsGO:00069249.4IL2RA, FAS
4inflammatory cell apoptotic processGO:00069259.3FASLG, FAS
5necroptotic signaling pathwayGO:00975279.3FASLG, FAS
6regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:020012399.2FAS, FASLG
7extrinsic apoptotic signaling pathwayGO:00971919.1FAS, FASLG
8extrinsic apoptotic signaling pathway via death domain receptorsGO:00086259.1FAS, FASLG
9apoptotic signaling pathwayGO:00971909.0FASLG, FAS
10activation of cysteine-type endopeptidase activity involved in apoptotic processGO:00069198.9FAS, FASLG
11immune responseGO:00069558.9IL2RA, FASLG, HLA-DRB1
12apoptotic processGO:00069158.3IL2RA, FASLG, FAS

Molecular functions related to Vogt-Koyanagi-Harada Disease according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1kinase bindingGO:00199009.3FAS, 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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet