- 関
- ヒト白血球抗原、大動脈炎症候群
WordNet
- the 2nd letter of the Roman alphabet (同)b
- the blood group whose red cells carry the B antigen (同)type_B, group B
- the 8th letter of the Roman alphabet (同)h
PrepTutorEJDIC
- hydrogenの化学記号
- 鉛筆の硬度 / 《俗》heroin
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2012/12/06 12:32:15」(JST)
[Wiki en表示]
|
HLA-B (alpha)-β2MG with bound peptide |
major histocompatibility complex (human), class I, B39
|
Alleles |
B*3901, 3902, 3903, . . |
Structure (See HLA-B) |
Shared data |
Locus |
chr.6 6p21.31 |
HLA-B39 (B39) is an HLA-B serotype. The serotype identifies the more common HLA-B*39 gene products.[1]
Further information: HLA-serotype tutorial
B39 is a split antigen of the broad antigen B16, and is a sister type of B38. B39 is most commonly found on the west pacific rim, in Japan and highest frequency in the new world. In Europe it is found in Scandinavia and Northern Russia.
Contents
- 1 Serotype
- 2 Alleles
- 3 Disease
- 3.1 In Takayasu's arteritis
- 4 References
|
Serotype
Serotypes B39, B16, and B38 recognition of the
HLA B*39 gene products[2]
B*39 |
B39 |
B16 |
B38 |
Sample |
allele |
% |
% |
% |
size (N) |
*3901 |
95 |
|
|
476 |
*3902 |
87 |
|
|
96 |
*3903 |
77 |
|
|
20 |
*3905 |
82 |
|
|
245 |
*3906 |
94 |
|
|
508 |
*3908 |
48 |
|
|
57 |
*3909 |
97 |
|
|
13 |
*3910 |
82 |
|
|
127 |
*3911 |
69 |
|
|
16 |
Alleles link-out to IMGT/HLA Databease at EBI |
The serology for the most common B39 alleles, B*3901 and B*3906 is good, but some allele products are not well detected. Given the differential involvement of these alleles in disease testing should involve high resolution typing.
HLA B*3906 frequencies
|
|
freq |
ref. |
Population |
(%) |
[3] |
Venezuela Perja Mountain Bari |
23.9 |
|
Mexico Mixtec Oaxaca |
8.8 |
|
USA Arizona Pima |
7.3 |
|
USA South Texas Hispanics |
5.6 |
|
Mexico Zaptotec Oaxaca |
4.5 |
|
New Mexico Canoncito Navajo |
3.7 |
|
Oman |
2.5 |
|
PNG Wanigela |
2.3 |
|
Tunisia Tunis |
2.3 |
|
North American Natives |
1.9 |
|
USA Hispanic |
1.7 |
|
Brazil Belo Horizonte |
1.6 |
|
South Dakota Lakota Sioux |
1.5 |
|
Cuban White |
1.4 |
|
Azores S. Maria & S. Miguel |
1.3 |
|
Australia New South Wales |
1.1 |
|
Finland |
1.1 |
|
Portugal North |
1.1 |
|
Portugal Centre |
1.0 |
|
Spain Eastern Andalusia Gipsy |
1.0 |
|
Brazil Terena |
0.9 |
|
Ireland Northern |
0.9 |
|
Azores Terceira Island |
0.8 |
|
USA Caucasian pop2 |
0.8 |
|
Brazil |
0.7 |
|
USA Philadelphia Caucasians |
0.7 |
|
India Andhra Pradesh Golla |
0.5 |
|
Thailand |
0.4 |
Alleles
HLA B*3901 frequencies
|
|
freq |
ref. |
Population |
(%) |
[3] |
Taiwan Saisiat |
54.9 |
[3] |
Taiwan Tsou |
24.5 |
[3] |
South Dakota Lakota Sioux |
22.5 |
[3] |
Taiwan Taroko |
21.8 |
[3] |
Taiwan Atayal |
19.8 |
[3] |
PNG Wanigela |
16.7 |
[3] |
Japan Ainu Hokkaido |
16.0 |
[3] |
Taiwan Bunun |
14.9 |
[3] |
New Mexico Canoncito Navajo |
14.6 |
[3] |
Taiwan Thao |
13.3 |
[3] |
Taiwan Rukai |
13.0 |
[3] |
Taiwan Ami |
10.2 |
[3] |
USA Hawaii Okinawa |
7.7 |
[3] |
Papua New Guinea Wosera |
7.0 |
[3] |
Papua New Guinea Madang |
6.4 |
[3] |
Mexico Mixtec Oaxaca |
5.9 |
[3] |
Taiwan Puyuma |
5.0 |
[3] |
Taiwan Paiwan |
4.9 |
[3] |
New Caledonia |
4.8 |
[3] |
Japan Central |
4.4 |
[3] |
American Samoa |
4.0 |
[3] |
USA North American Natives |
4.0 |
[3] |
Taiwan Hakka |
3.6 |
[3] |
Indig. Australian Cape York Peninsula |
3.5 |
[3] |
Japan (5) |
3.5 |
[3] |
Philippines Ivatan |
3.0 |
[3] |
Brazil |
2.9 |
[3] |
China Guangxi Maonan |
2.8 |
[3] |
Georgia Tibilisi Georgians |
2.8 |
[3] |
China Yunnan Nu |
2.6 |
[3] |
Thailand |
2.5 |
[3] |
Azores Terceira Island |
2.4 |
[3] |
Singapore Chinese |
2.4 |
[3] |
Romanian |
2.3 |
[3] |
China Yunnan Lisu |
2.2 |
[3] |
Indig. Australian Yuendumu |
2.1 |
[3] |
China Guangzhou |
2.0 |
[3] |
Croatia |
2.0 |
[3] |
France South East |
1.9 |
[3] |
Georgia Svaneti Svans |
1.9 |
[3] |
PNG Karimui Plateau |
1.9 |
[3] |
Azores Central Islands |
1.8 |
[3] |
Spain Eastern Andalusia |
1.8 |
[3] |
Uganda Kampala |
1.6 |
[3] |
Mexico Zaptotec Oaxaca |
1.5 |
[3] |
Singapore Thai |
1.5 |
[3] |
China Guangzhou Han |
1.4 |
[3] |
China Qinghai Hui |
1.4 |
[3] |
Czech Republic |
1.4 |
[3] |
Madeira |
1.4 |
[3] |
Indig. Australian Kimberly |
1.3 |
[3] |
Finland |
1.1 |
Disease
B39 is suggested as a factor in Takayasu's arteritis and gallstones in Mexico.[4] Osteoarticular complications of brucellosis appear to be associated with B39.[5] An association with spondylarthropathies [6][7] and psoriatic arthritis[8][9] was observed in several studies. Psoriatic arthritis appears to be linked to MICA-A9 which tightly linked to HLA-B39.[10][11] B39 also appears to be involved in the Fishers syndrome variant of Guillain-Barré syndrome.[12]
B39 appears to be protective against cardiomyopathy in Chaga's disease indicating a possible selective factor in its rise in the New World.[13] Chaga's disease is caused by a trypanosome carried by a blood sucking insect found in tropical, palm growing regions.
Southern California now reports cases of Chaga's disease from contaminated transfusions and may be already a habitat for the vector.[14]
In Takayasu's arteritis
Takayasu's arteritis appears to have a link to B39.[15][16] The association with B*3902 increases risk of pulmonary infarction, ischemic heart disease, aortic regurgitation, systemic hypertension, renal artery stenosis, cerebrovascular disease, and visual disturbance.[17] B*3906, common in indigenous Mesoamericans has been found associated with the same disease.[18]
HLA B*3902 frequencies
|
|
freq |
ref. |
Population |
(%) |
[3] |
Mexico Mixe Oaxaca |
38.7 |
|
Mexico Zaptotec Oaxaca |
13.4 |
|
Mexico Mixtec Oaxaca |
5.9 |
|
Mexico Mestizos |
1.2 |
|
Japan pop5 |
0.9 |
|
Cuban White |
0.7 |
|
Israel Ashk. and Non Ashk. Jews |
0.5 |
|
Japan Central |
0.5 |
|
Senegal Niokholo Mandenka |
0.5 |
B*3903 |
|
PNG New Britain Rabaul |
13.2 |
|
Brazil Terena |
11.2 |
|
Argentina Toba Rosario |
5.2 |
|
Brazil |
0.7 |
|
Finland |
0.6 |
|
Kenya Luo |
0.6 |
|
North American Natives |
0.5 |
B*3904 |
|
Argentina Toba Rosario |
1.2 |
|
Georgia Svaneti Svans |
0.6 |
|
Jordan Amman |
0.3 |
|
Shijiazhuang Tianjian Han |
0.1 |
|
Japan Central |
0.1 |
|
Romanian |
0.1 |
B*3905 |
|
Venezuela Perija Yucpa |
36.1 |
|
Mexico Zaptotec Oaxaca |
12.7 |
|
Mexico Mixtec Oaxaca |
9.8 |
|
Mexico Mestizos |
4.9 |
|
Arizona Pima |
4.5 |
|
Mexico Guadalajara Mestizos |
2.4 |
|
Argentina Toba Rosario |
2.3 |
|
USA Hispanic |
2.1 |
|
Mexico Mixe Oaxaca |
1.9 |
|
Cuban White |
1.4 |
|
Mexico Chihuahua Tarahumara |
1.1 |
|
USA North American Natives |
0.5 |
|
Shijiazhuang Tianjian Han |
0.2 |
B*3907 |
|
Shijiazhuang Tianjian Han |
0.6 |
|
Singapore Thai |
0.5 |
|
China South Han |
0.2 |
B*3908 |
|
Mexico Zaptotec Oaxaca |
2.2 |
|
Mexico Mestizos |
1.2 |
|
Mexico Mixtec Oaxaca |
1.0 |
|
Brazil |
0.7 |
|
USA Hispanic |
0.6 |
B*3909 |
|
Venezuela Perija Yucpa |
34.9 |
|
Thailand pop3 |
3.1 |
|
Brazil Terena |
1.7 |
|
China South Han |
1.4 |
|
Argentina Toba Rosario |
1.2 |
|
China Qinghai Hui |
0.9 |
B*3910 |
|
Sudanese |
2.5 |
|
Senegal Niokholo Mandenka |
2.1 |
|
South African Natal Zulu |
1.5 |
|
Cameroon Beti |
1.4 |
|
Spain Eastern Andalusia |
1.2 |
|
Kenya Luo |
1.1 |
|
Israel Arab Druse |
1.0 |
|
Kenya Nandi |
1.0 |
|
Guinea Bissau |
0.8 |
|
Kenya |
0.7 |
|
Mali Bandiagara |
0.7 |
|
Morocco Nador Metalsa Class I |
0.7 |
|
Cameroon Bamileke |
0.6 |
|
Cameroon Yaounde |
0.5 |
|
Israel Ashk. and Non Ashk. Jews |
0.5 |
|
Saudi Arabia Guraiat and Hail |
0.5 |
References
- ^ Marsh SG, Albert ED, Bodmer WF, et al. (2005). "Nomenclature for factors of the HLA system, 2004". Tissue Antigens 65 (4): 301–69. doi:10.1111/j.1399-0039.2005.00379.x. PMID 15787720.
- ^ derived from IMGT/HLA
- ^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa ab ac ad ae af ag ah ai aj ak al am an ao ap aq ar as at au av aw ax ay az ba bb Middleton D, Menchaca L, Rood H, Komerofsky R (2003). "New allele frequency database: http://www.allelefrequencies.net". Tissue Antigens 61 (5): 403–7. doi:10.1034/j.1399-0039.2003.00062.x. PMID 12753660.
- ^ Méndez-Sánchez N, King-Martínez AC, Ramos MH, Pichardo-Bahena R, Uribe M (November 2004). "The Amerindian's genes in the Mexican population are associated with development of gallstone disease". Am. J. Gastroenterol. 99 (11): 2166–70. doi:10.1111/j.1572-0241.2004.40159.x. PMID 15554998.
- ^ Bravo MJ, Colmenero Jde D, Alonso A, Caballero A (May 2003). "HLA-B*39 allele confers susceptibility to osteoarticular complications in human brucellosis". J. Rheumatol. 30 (5): 1051–3. PMID 12734905.
- ^ Sobao Y, Tsuchiya N, Takiguchi M, Tokunaga K (January 1999). "Overlapping peptide-binding specificities of HLA-B27 and B39: evidence for a role of peptide supermotif in the pathogenesis of spondylarthropathies". Arthritis Rheum. 42 (1): 175–81. doi:10.1002/1529-0131(199901)42:1<175::AID-ANR21>3.0.CO;2-7. PMID 9920028.
- ^ Alvarez I, López de Castro JA (July 2000). "HLA-B27 and immunogenetics of spondyloarthropathies". Curr Opin Rheumatol 12 (4): 248–53. doi:10.1097/00002281-200007000-00003. PMID 10910175. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=1040-8711&volume=12&issue=4&spage=248.
- ^ Eastmond CJ (May 1994). "Psoriatic arthritis. Genetics and HLA antigens". Baillieres Clin Rheumatol 8 (2): 263–76. doi:10.1016/S0950-3579(94)80018-9. PMID 8076387.
- ^ Gladman DD, Farewell VT (June 1995). "The role of HLA antigens as indicators of disease progression in psoriatic arthritis. Multivariate relative risk model". Arthritis Rheum. 38 (6): 845–50. doi:10.1002/art.1780380619. PMID 7779129.
- ^ Bolognesi E, Dalfonso S, Rolando V, Fasano ME, Praticò L, Momigliano-Richiardi P (October 2001). "MICA and MICB microsatellite alleles in HLA extended haplotypes". Eur. J. Immunogenet. 28 (5): 523–30. doi:10.1046/j.0960-7420.2001.00250.x. PMID 11881819. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0960-7420&date=2001&volume=28&issue=5&spage=523.
- ^ González S, Martínez-Borra J, López-Vázquez A, García-Fernández S, Torre-Alonso JC, López-Larrea C (May 2002). "MICA rather than MICB, TNFA, or HLA-DRB1 is associated with susceptibility to psoriatic arthritis". J. Rheumatol. 29 (5): 973–8. PMID 12022360. http://www.jrheum.com/subscribers/02/05/973.html.
- ^ Yuki N, Sato S, Tsuji S, Ogawa K, Miyatake T (June 1993). "Human leukocyte antigens in Fisher's syndrome". Ann. Neurol. 33 (6): 655–7. doi:10.1002/ana.410330617. PMID 8498847.
- ^ Cruz-Robles D, Reyes PA, Monteón-Padilla VM, Ortiz-Muñiz AR, Vargas-Alarcón G (January 2004). "MHC class I and class II genes in Mexican patients with Chagas disease". Hum. Immunol. 65 (1): 60–5. doi:10.1016/j.humimm.2003.10.008. PMID 14700597. http://linkinghub.elsevier.com/retrieve/pii/S0198885903006360.
- ^ http://www.medscape.com/viewarticle/565341
- ^ Kimura A, Kitamura H, Date Y, Numano F (August 1996). "Comprehensive analysis of HLA genes in Takayasu arteritis in Japan". Int. J. Cardiol. 54 Suppl: S61–9. PMID 9119528.
- ^ Yoshida M, Kimura A, Katsuragi K, Numano F, Sasazuki T (August 1993). "DNA typing of HLA-B gene in Takayasu's arteritis". Tissue Antigens 42 (2): 87–90. doi:10.1111/j.1399-0039.1993.tb02242.x. PMID 7903491.
- ^ Kitamura H, Kobayashi Y, Kimura A, Numano F (October 1998). "Association of clinical manifestations with HLA-B alleles in Takayasu arteritis". Int. J. Cardiol. 66 Suppl 1: S121–6. doi:10.1016/S0167-5273(98)00159-4. PMID 9951811.
- ^ Rodríguez-Reyna TS, Zúñiga-Ramos J, Salgado N, et al. (October 1998). "Intron 2 and exon 3 sequences may be involved in the susceptibility to develop Takayasu arteritis". Int. J. Cardiol. 66 Suppl 1: S135–8; discussion S139. doi:10.1016/S0167-5273(98)00161-2. PMID 9951813.
HLA-B serotypes and allele groups
|
|
- HLA-B
- B5
- B7
- B8
- B12
- B13
- B14
- B15
- B62
- B63
- B70
- B71
- B72
- B75
- B76
- B77
- B16
- B17
- B18
- B21
- B22
- B27
- B35
- B37
- B40
- B41
- B42
- B46
- B47
- B48
- B53
- B59
- B67
- B73
- B78
- B81
- B*82
- B*83
|
|
UpToDate Contents
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English Journal
- Cutaneous polyarteritis nodosa associated with HLA-B39-positive undifferentiated spondyloarthritis in a Japanese patient.
- Nakano H, Ooka S, Shibata T, Ogawa H, Ito H, Takakuwa Y, Tonooka K, Maeda A, Yamasaki Y, Kiyokawa T, Nagafuchi H, Yamada H, Ozaki S.SourceDivision of Rheumatology and Allergy, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa 216-8511, Japan. hiro-n@marianna-u.ac.jp
- Modern rheumatology / the Japan Rheumatism Association.Mod Rheumatol.2012 Sep;22(5):783-6. doi: 10.1007/s10165-011-0576-7. Epub 2012 Jan 21.
- We present the case of a 43-year-old man diagnosed with HLA-B39-positive spondyloarthritis who developed cutaneous lesions consistent with cutaneous polyarteritis nodosa (CPN). Previous studies indicated an elevated incidence of HLA-B39 in HLA-B27-negative Japanese patients with spondyloarthritis. T
- PMID 22270344
- New human leukocyte antigen risk allele in Japanese patients with Takayasu arteritis.
- Takamura C, Ohhigashi H, Ebana Y, Isobe M.SourceDepartment of Cardiovascular Medicine, Tokyo Medical and Dental University, Tokyo, Japan.
- Circulation journal : official journal of the Japanese Circulation Society.Circ J.2012;76(7):1697-702. Epub 2012 May 16.
- BACKGROUND: The association of human leukocyte antigen (HLA) alleles and Takayasu arteritis (TA) is not fully understood. The aim of the present study was to investigate HLA alleles in Japanese patients with TA and the association of these alleles with clinical manifestations.METHODS AND RESULTS: A
- PMID 22664757
Japanese Journal
- A Family with Cases of Adult Onset Still's Disease and Psoriatic Arthritis
- MAEDA Hiroyuki,KONISHI Futoshi,HIYAMA Keiko,ISHIOKA Shinichi,YAMAKIDO Michio
- Internal medicine 39(1), 77-79, 2000-01-01
- … On the other hand, the association between HLA subtypes and several groups of seronegative arthritis including psoriatic arthritis has been well documented. … Both patients were HLA-B39-positive, which was likely playing important pathogenic roles in the latter case. … Clinical and immunological aspects of HLA-B39-related inflammatory diseases are also discussed.(Internal Medicine 39: 77-79, 2000) …
- NAID 10013261199
Related Links
- HLA-DRw53, 4.5. 原発性胆汁性肝硬変, HLA-DR8(DRB1*08:03), 2.2. HLA-DR2( DRB1*16:02), 5.9. 全身性エリテマトーデス, HLA-B39, 6.3. HLA-DR2(DRB1*15: 01), 3.0. Crohn病, HLA-DRB1*04:05, 2.0. HLA-DQB1*04:01, 2.0. 潰瘍性大腸炎 ...
- HLA-B39 (B39) is an HLA-B serotype. The serotype identifies the more common HLA-B*39 gene .... Psoriatic arthritis appears to be linked to MICA-A9 which tightly linked to HLA-B39. B39 also appears to be involved in the Fishers syndrome ...
Related Pictures
★リンクテーブル★
[★]
- 英
- aortitis syndrome
- 同
- 高安動脈炎 Takayasu arteritis Takayasu's arteritis TA、高安病 Takayasu's disease Takayasu disease、脈なし病 pulseless disease、高安閉塞症 Takayasu occlusive disease、特発性肉芽腫性動脈炎 idiopathic granulomatousarteritis
- 関
- 難病、血管炎
概念
病因
疫学
- 日本が世界的に多く、アジア諸国(インド、中国など)、ソ連、メキシコ、南アフリカでもみられる。(参考3)
- 1.2-2.6/100万人 (HIM.2127)
- 若年者。 10-40歳(PHD.360)
- 女性に多い
- 男:女=1:10 1:9(IMD YN.C-151)
我が国が世界でもっとも多いといわれている。でも少なくないようである。←日本語の意味が分からない
病理
- 大動脈や分岐幹動脈など弾性型動脈が侵される。大動脈弓から下行大動脈にかけて好発する。とくに腕頭動脈、総頚動脈、鎖骨下動脈の分岐部に多い。(参考3)
- 病変は外膜から進行し、中膜がもっとも強く侵される。動脈壁全層が肥厚し、内腔は狭窄にて閉塞する。(参考3)
- 線維化をともなう肉芽腫性血管炎。管腔の狭小化が顕著。(PHD.361)
- 内膜の過形成。中膜、外膜の肥厚。慢性期には線維化と内腔の狭窄。(HIM.1567)
- there are marked intimal proliferation and fibrosis, scarring and vascularization of the media, and disruption and degeneration of the elastic lamina.(HIM.2127)
- 炎症性の単核球の浸潤がみられ、時に巨核球が見られる。(HIM.2127)
- 連続・斑状の肉芽腫性炎症が見られ、リンパ球、組織球、多核巨細胞が見られる(PHD.361) → 内膜の増殖、男性板の破壊、線維化
症状
- 早期には全身症状、慢性期には血管内腔狭窄・閉塞による症状が出る
- 全身症状:発熱、盗汗、全身倦怠感、食欲不振、関節痛、体重減少
- 血管狭窄・閉塞による症状:上肢の跛行、脳虚血、失神、(時に)狭心症
- 皮膚症状:結節性紅斑、壊死性膿皮症
閉塞部位と症状 HIM.2127
table 319-6 Frequency of Arteriographic Abnormalities and Potential Clinical Manifestations of Arterial Involvement in Takayasu's Arteritis
|
動脈
|
動脈造影状の異常(%)
|
来しうる症状
|
鎖骨下動脈
|
93
|
上肢の跛行(arm claudication)、レイノー症候群
|
総頚動脈
|
58
|
視覚の変化、失神、TIA、脳梗塞
|
腹部大動脈
|
47
|
腹痛、悪心、嘔吐
|
腎動脈
|
38
|
高血圧、腎不全
|
大動脈弓/大動脈根部
|
35
|
大動脈弁閉鎖不全症、うっ血性心不全
|
椎骨動脈
|
35
|
視覚の変化(visual change)、めまい
|
腹腔動脈
|
18
|
腹痛、悪心、嘔吐
|
上腸間膜動脈
|
18
|
腹痛、悪心、嘔吐
|
腸骨動脈
|
17
|
下肢の跛行(leg claudication)
|
肺動脈
|
10?40
|
非定型的な胸痛(atypical chest pain)、呼吸困難
|
冠状動脈
|
<10
|
胸痛、心筋梗塞
|
資料3
表1 大動脈炎症候群の症状出現頻度
|
症状
|
頻度(%)
|
脳循環障害(めまい、頭痛、失神)
|
60
|
眼症状
|
20
|
心症状(狭心症様症状)
|
60
|
高血圧
|
50
|
四肢循環障害(しびれ、冷感、脈拍欠損)
|
70
|
全身症状(発熱、倦怠感)
|
70
|
身体所見
- 脈を触れない
- 血圧の左右差、上下肢差(下肢>>上肢) ← 鎖骨下動脈が冒される事による
- 血管雑音 ← 血管内腔の狭窄による
- 高血圧:32-93%の患者で(HIM. 2127)
- (古典的?)脈なし病の三徴:(1)上肢の脈を触れない、(2)眼底変化(後期の変化に花冠状動静脈吻合、花環状動静脈吻合)、(3)頚動脈反射亢進(参考4)
- 上半身に脈を触れず、進行すると頚動脈反射亢進により頭部を後屈させると脈拍数減少、血圧低下、失神、過呼吸をきたす。緩徐に進行する血圧低下に伴う血流速度低下により網膜血管が拡張、また毛細血管瘤が耳側周辺部から眼底全体に広がる。視神経乳頭を取り巻いて馬蹄形または花環状の動静脈吻合が形成されるようになる。(SOP.144)
検査
- ESR上昇、CRP上昇
- 単純CT:全周性に血管壁が肥厚。肥厚した血管壁に高吸収を認めることがある。
- 造影CT:中膜から外膜にかけて造影効果あり。内膜は造影されない。
診断
- 参考1-1
- 1. 確定診断は画像診断(DSA,CT,MRA)によって行う。
- 2. 若年者で血管造影によって大動脈とその第一次分枝に閉塞性あるいは拡張性病変を多発性に認めた場合は、炎症反応が陰性でも大動脈炎症候群(高安動脈炎)を第1に疑う。
- 3. これに炎症反応が陽性ならば、大動脈炎症候群(高安動脈炎)と診断する。
- 4. 上記の自覚症状、検査所見を有し、下記の鑑別疾患を否定できるもの。
鑑別診断
合併症
- 大動脈弁閉鎖とそれによる心不全、大動脈瘤、腎血管性高血圧、膠原病 (YN.C-152)
- 骨関節病変、慢性甲状腺炎 (YN.C-152)
治療
薬物療法
外科療法&カテーテルインターベンション
外科療法
死因
- うっ血性心不全、心筋梗塞、脳梗塞・脳出血、腎不全、肺梗塞 (YN.C-151)
予後
参考
- http://www.nanbyou.or.jp/entry/290
- http://www.nanbyou.or.jp/upload_files/065_s.pdf
- http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf
- http://www.nanbyo.or.jp/zaidan/nanbyo/tokuteisikkan_list/13/content13.html
- 4. 2.螢光眼底血管造影法による脈なし病網膜血行動態の観察と,本病網膜血管病変の組織学的研究 生井浩 松井弘治
- http://journal.akademi.jp/jca/pdf/19701005/jca_1970_1005_0253_0.pdf
[★]
- 英
- human leucocyte antigen, HLA
- 同
- ヒト白血球型抗原、HLA抗原 HLA antigen、ヒト組織適合性白血球抗原 human histocompatibility leukocyteantigen
- 主要組織適合抗原 MHC ← 免疫との関連はこちらを参照
- 関
- 骨髄バンク、HLA抗原
疾患との関連
出典不明
強直性脊椎炎
|
B27
|
関節リウマチ
|
DR4
|
重症筋無力症
|
DR9,DQ3
|
尋常性天疱瘡
|
A26,DR4
|
バセドウ病
|
DR5
|
I型糖尿病(インスリン依存性糖尿病)
|
B54,DR4,DR9,DR53,DQ4
|
グレーブス病
|
DR5
|
ベーチェット病
|
B51
|
原田病
|
DR4,DR53
|
潰瘍性大腸炎
|
B52,DR2
|
クローン病
|
DR4,DQ3
|
高安病
|
B52,DR2,DQ1
|
バージャー病
|
B52,DR2,DQ1
|
ナルコレプシー
|
DR2
|
first aid step1 2006 p.191
まとめ
[★]
- Mg2+存在下でC3, B, Dが反応してC3bBbとなり、これがC3転換酵素(C3bBb)あるいはC5転換酵素(C3bBb3b)を形成する。これらはP(properdin)と結合して活性化し、それぞれC3、C5を活性化する
[★]
[★]
[★]
ヒト白血球抗原 human leukocyte antigen