DISEASE: Behcet disease


Entry
H01476                      Disease                                
Name
Behcet disease;
Behcet syndrome
Description
Behcet disease is a multisystemic inflammatory disease characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, other skin lesions, and uveitis. It can also involve visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, cardiovascular and neurological systems. This disease is more common in countries along the ancient Silk Road, including Asia, Middle East, and Mediterranean. Although the etiology is still unknown, this disease is believed to be triggered by environmental factors such as microbial agents in individuals with a particular genetic background. The positive association of HLA-B51 was identified more than four decades ago, and has been confirmed in multiple populations. Recent genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behcet disease.
Category
Immune system disease; Skin disease
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H01476  Behcet disease
 Skin diseases
  Skin and soft tissue diseases
   H01476  Behcet disease
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Autoinflammatory disorders
   4A62  Beh?et disease
    H01476  Behcet disease
Related
pathway
hsa04630  JAK-STAT signaling pathway
hsa04060  Cytokine-cytokine receptor interaction
Gene
HLA-B (polymorphism) [HSA:3106] [KO:K06751]
IL10 (polymorphism) [HSA:3586] [KO:K05443]
IL23R (polymorphism) [HSA:149233] [KO:K05065]
IL12RB2 (polymorphism) [HSA:3595] [KO:K05064]
Drug
Apremilast [DR:D08860]
Other DBs
ICD-11: 4A62
ICD-10: M35.2
MeSH: D001528
OMIM: 109650
Reference
PMID:16282155
  Authors
Bonfioli AA, Orefice F
  Title
Behcet's disease.
  Journal
Semin Ophthalmol 20:199-206 (2005)
DOI:10.1080/08820530500231953
Reference
PMID:25062414
  Authors
Demirseren DD, Ceylan GG, Akoglu G, Emre S, Erten S, Arman A, Metin A
  Title
HLA-B51 subtypes in Turkish patients with Behcet's disease and their correlation with clinical manifestations.
  Journal
Genet Mol Res 13:4788-96 (2014)
DOI:10.4238/2014.July.2.8
Reference
PMID:26347074
  Authors
Takeuchi M, Kastner DL, Remmers EF
  Title
The immunogenetics of Behcet's disease: A comprehensive review.
  Journal
J Autoimmun 64:137-48 (2015)
DOI:10.1016/j.jaut.2015.08.013

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Disease (1)   
   OMIM (1)   
Drug (16)   
   KEGG DRUG (16)   
Gene (8)   
   KEGG ORTHOLOGY (4)   
   KEGG GENES (4)   
Literature (3)   
   PubMed (3)   
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