| H01698 | |
| H番号 | H01698 |
| 名称 | 巨細胞性動脈炎; 側頭動脈炎 |
| 概要 | 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. |
| カテゴリ | 免疫系疾患 |
| ネットワーク | - |
| 病因遺伝子 | HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752] PTPN22 (polymorphism) [HSA:26191] [KO:K18024] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | トシリズマブ [DR:D02596] |
| コメント | See also H01465 Large-vessel vasculitis (LVV). |
| リンク | ICD-11: 4A44.2 ICD-10: M31.6 MeSH: D013700 OMIM: 187360 |
| 文献 | PMID:27708947 著者 Gracanin AG, Curic J, Loncarevic J, Morovic-Vergles J タイトル Magnetic resonance imaging in the diagnosis and follow-up of giant cell arteritis: case report and review of literature. 雑誌 Eur J Rheumatol 2:125-128 (2015) DOI:10.5152/eurjrheum.2015.0080 PMID:25817017 著者 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 タイトル A large-scale genetic analysis reveals a strong contribution of the HLA class II region to giant cell arteritis susceptibility. 雑誌 Am J Hum Genet 96:565-80 (2015) DOI:10.1016/j.ajhg.2015.02.009 PMID:26819753 著者 Evans J, Steel L, Borg F, Dasgupta B タイトル Long-term efficacy and safety of tocilizumab in giant cell arteritis and large vessel vasculitis. 雑誌 RMD Open 2:e000137 (2016) DOI:10.1136/rmdopen-2015-000137 |