PMID- 26068404
OWN - NLM
STAT- MEDLINE
DCOM- 20160324
LR  - 20220408
IS  - 1863-2300 (Electronic)
IS  - 1863-2297 (Linking)
VI  - 37
IP  - 4
DP  - 2015 Jul
TI  - Pathogenesis of Behcet's disease: autoinflammatory features and beyond.
PG  - 413-8
LID - 10.1007/s00281-015-0502-8 [doi]
AB  - Behcet's disease (BD) is an inflammatory disorder of unknown aetiology 
      characterised by recurrent attacks affecting the mucocutaneous tissues, eyes, 
      joints, blood vessels, brain and gastrointestinal tract. It is a multifactorial 
      disease classified as a variable vessel vasculitis, and several environmental 
      triggers may induce inflammatory episodes in genetically susceptible individuals. 
      BD has several autoinflammatory features including recurrent self-limited 
      clinical manifestations overlapping with monogenic autoinflammatory disorders, 
      significant host predisposition and abnormally increased inflammatory response, 
      with a robust innate component. Human leukocyte antigen (HLA)-B*51 is the 
      strongest susceptibility factor described so far affecting the disease risk and 
      typical phenotype. Non-HLA genetic associations such as endoplasmic reticulum 
      aminopeptidase 1 (ERAP1), interleukin 23 receptor (IL23R) and IL10 variations 
      suggest that BD shares susceptibility genes and inflammatory pathways with 
      spondyloarthritis. Although genomewide association studies revealed an increased 
      risk associated with recessively inherited ERAP1 variations in HLA-B*51 positive 
      patients, it is not clear yet whether certain peptide-HLA allele combinations 
      result in an adaptive response by a self-antigen-directed cytotoxic response or 
      an innate response by modulating an NK cell activity or causing an unfolded 
      protein response. Understanding of major histocompatibility complex (MHC) Class 
      I-driven inflammatory response is expected to provide insights for the 
      development of better treatment and remission-induction options in BD as well as 
      in ankylosing spondylitis (AS) and psoriasis.
FAU - Gul, Ahmet
AU  - Gul A
AD  - Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of 
      Medicine, Istanbul University, 34093, Fatih, Istanbul, Turkey, 
      agul@istanbul.edu.tr.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20150612
PL  - Germany
TA  - Semin Immunopathol
JT  - Seminars in immunopathology
JID - 101308769
RN  - 0 (HLA-B51 Antigen)
SB  - IM
MH  - Behcet Syndrome/*diagnosis/drug therapy/*etiology
MH  - Disease Susceptibility
MH  - HLA-B51 Antigen/chemistry/genetics/immunology/metabolism
MH  - Hereditary Autoinflammatory Diseases/diagnosis/drug therapy/etiology
MH  - Humans
MH  - Inflammation/etiology/metabolism
EDAT- 2015/06/13 06:00
MHDA- 2016/03/25 06:00
CRDT- 2015/06/13 06:00
PHST- 2015/05/05 00:00 [received]
PHST- 2015/05/25 00:00 [accepted]
PHST- 2015/06/13 06:00 [entrez]
PHST- 2015/06/13 06:00 [pubmed]
PHST- 2016/03/25 06:00 [medline]
AID - 10.1007/s00281-015-0502-8 [doi]
PST - ppublish
SO  - Semin Immunopathol. 2015 Jul;37(4):413-8. doi: 10.1007/s00281-015-0502-8. Epub 
      2015 Jun 12.