KEGG   DISEASE: Giant cell arteritisHelp
Entry
H01698                      Disease                                

Name
Giant cell arteritis;
Temporal arteritis
  Supergrp
Large-vessel vasculitis [DS:H01465]
Description
Giant cell arteritis (GCA), also known as temporal arteritis, is a chronic and polygenic immune-mediated disease of unknown etiology. It is the most common form of vasculitis in individuals over the age of 50 in Western countries. It is characterized by inflammatory damage of the aorta and/or aortic branches, particularly the temporal artery, which can lead to severe complications such as blindness or cerebrovascular disorders. GCA is twice as common in women. It is frequently associated with polymyalgia rheumatica. Laboratory tests usually reveal high erythrocyte sedimentation rate (ESR), elevated levels of C-reactive protein (CRP) and other acute phase proteins, anemia of chronic disease, and thrombocytosis. Genetic association studies have described several genes that are associated with predisposition to GCA, including genes of immune-inflammatory pathways and genes of the HLA class I and II regions. The HLA-DRB1*04 alleles seem to be the most consistently associated genetic risk factors for GCA. Outside the HLA region, the most significant loci included PTPN22. Glucocorticoids are currently the mainstay of treatment for GCA but are associated with frequent adverse events. Tocilizumab, a humanised antihuman IL-6 receptor antibody, has been used successfully in several reports as a treatment of GCA.
Category
Immune system disease; Cardiovascular disease; Nervous system disease
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Other immune system diseases
   H01698  Giant cell arteritis
 Cardiovascular diseases
  Vascular diseases
   H01698  Giant cell arteritis
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A44  Vasculitis
    H01698  Giant cell arteritis
BRITE hierarchy
Gene
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
PTPN22 (polymorphism) [HSA:26191] [KO:K18024]
Drug
Triamcinolone acetonide [DR:D00983]
Dexamethasone [DR:D00292]
Hydrocortisone sodium succinate [DR:D00978]
Prednisolone sodium phosphate [DR:D00981]
Methylprednisolone sodium succinate [DR:D00751]
Methylprednisolone acetate [DR:D00979]
Tocilizumab [DR:D02596]
Comment
See also H01465 Large-vessel vasculitis (LVV).
Other DBs
ICD-11: 4A44.2
ICD-10: M31.6
MeSH: D013700
OMIM: 187360
Reference
PMID:27708947 (marker)
  Authors
Gracanin AG, Curic J, Loncarevic J, Morovic-Vergles J
  Title
Magnetic resonance imaging in the diagnosis and follow-up of giant cell arteritis: case report and review of literature.
  Journal
Eur J Rheumatol 2:125-128 (2015)
DOI:10.5152/eurjrheum.2015.0080
Reference
  Authors
Carmona FD, Mackie SL, Martin JE, Taylor JC, Vaglio A, Eyre S, Bossini-Castillo L, Castaneda S, Cid MC, Hernandez-Rodriguez J, Prieto-Gonzalez S, Solans R, Ramentol-Sintas M, Gonzalez-Escribano MF, Ortiz-Fernandez L, Morado IC, Narvaez J, Miranda-Filloy JA, Beretta L, Lunardi C, Cimmino MA, Gianfreda D, Santilli D, Ramirez GA, Soriano A, Muratore F, Pazzola G, Addimanda O, Wijmenga C, Witte T, Schirmer JH, Moosig F, Schonau V, Franke A, Palm O, Molberg O, Diamantopoulos AP, Carette S, Cuthbertson D, Forbess LJ, Hoffman GS, Khalidi NA, Koening CL, Langford CA, McAlear CA, Moreland L, Monach PA, Pagnoux C, Seo P, Spiera R, Sreih AG, Warrington KJ, Ytterberg SR, Gregersen PK, Pease CT, Gough A, Green M, Hordon L, Jarrett S, Watts R, Levy S, Patel Y, Kamath S, Dasgupta B, Worthington J, Koeleman BP, de Bakker PI, Barrett JH, Salvarani C, Merkel PA, Gonzalez-Gay MA, Morgan AW, Martin J
  Title
A large-scale genetic analysis reveals a strong contribution of the HLA class II region to giant cell arteritis susceptibility.
  Journal
Am J Hum Genet 96:565-80 (2015)
DOI:10.1016/j.ajhg.2015.02.009
Reference
PMID:26819753 (drug)
  Authors
Evans J, Steel L, Borg F, Dasgupta B
  Title
Long-term efficacy and safety of tocilizumab in giant cell arteritis and large vessel vasculitis.
  Journal
RMD Open 2:e000137 (2016)
DOI:10.1136/rmdopen-2015-000137
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