Takayasu arteritis

高安動脈炎

WordNet

1. inflammation of an artery

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/06 18:31:46」(JST)

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Takayasu's arteritis
Left anterior oblique angiographic image of Takayasu's arteritis showing areas of stenosis in multiple great vessels
Classification and external resources
Specialty	rheumatology
ICD-10	M31.4
ICD-9-CM	446.7
OMIM	207600
DiseasesDB	12879
MedlinePlus	001250
eMedicine	med/2232 ped/1956 neuro/361 radio/51
MeSH	D013625

Takayasu's disease (also known as "aortic arch syndrome", "nonspecific aortoarteritis" and the "pulseless disease") is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, affecting often young or middle-aged women of Asian descent. It mainly affects the aorta (the main blood vessel leaving the heart) and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males. Those with the disease often notice symptoms between 15 and 30 years of age. In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's arteritis. Takayasu's arteritis is similar to other forms of vasculitis, including giant cell arteritis which typically affects older individuals. Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, Takayasu's arteritis can present as pulseless upper extremities (arms, hands, and wrists with weak or absent pulses on the physical examination) which may be why it is also commonly referred to as the "pulseless disease". Involvement of renal arteries may lead to presentation as renovascular hypertension.

Sign and symptoms

Some people develop an initial "inflammatory phase" characterized by systemic illness with signs and symptoms of malaise, fever, night sweats, weight loss, joint pain, fatigue, and fainting. Fainting may result from subclavian steal syndrome or carotid sinus hypersensitivity. There is also often anemia and marked elevation of the ESR or C-reactive protein (nonspecific markers of inflammation). The initial "inflammatory phase" is often followed by a secondary "pulseless phase". The "pulseless phase" is characterized by vascular insufficiency from intimal narrowing of the vessels manifesting as arm or leg claudication, renal artery stenosis causing hypertension, and neurological manifestations due to decreased blood flow to the brain. Of note is the function of renal artery stenosis in causation of high blood pressure: Normally perfused kidneys produce proportionate amount of a substance called renin. Stenosis of the renal arteries causes hypo-perfusion (decreased blood flow) of the juxtaglomerular apparatus, resulting in exaggerated secretion of renin, and high blood levels of aldosterone, eventually leading to water and salt retention and high blood pressure. The neurological symptoms of the disease vary depending on the degree, and the nature of the blood vessel obstruction and can range from lightheadedness, to seizures in severe cases. One rare but important feature of the Takayasu's arteritis is ocular involvement in form of visual field defects, vision loss, or retinal hemorrhage. Some individuals with Takayasu's arteritis may present with only late vascular changes, without a preceding systemic illness. In the late stage, weakness of the arterial walls may give rise to localized aneurysms. As with all aneurysms, possibility of rupture and vascular bleeding is existent and requires monitoring. In view of chronic process and good collateral development, Raynaud's phenomenon or digital gangrene are very rare in Takayasu arteritis.

Pathophysiology

Axial T1-weighted post-gadolinium MRI in a patient with Takayasu arteritis showing thickened, enhancing aortic wall, consistent with large vessel vasculitis

Although its cause is unknown, the condition is characterized by segmental and patchy granulomatous inflammation of the aorta and its major derivative branches. This inflammation leads to arterial stenosis, thrombosis, and aneurysms. There is also irregular fibrosis of the blood vessels due to chronic vasculitis, leading to sometimes massive intimal fibrosis (fibrosis of the inner section of the blood vessels). Prominent narrowing due to inflammation, granuloma, and fibrosis is often seen in arterial studies such as magnetic resonance angiography (MRA), computed tomography angiography (CTA), or arterial angiography (DSA).

The genetic contribution to the pathogenesis of Takayasu's arteritis is supported by the genetic association with HLA-B∗52. A recent large collaborative study uncovered multiple additional susceptibility loci for this disease, increasing the number of genetic loci for this disease to five risk loci across the genome. About 200,000 genetic variants were genotyped in two ethnically divergent Takayasu's arteritis cohorts from Turkey and North America by using a custom-designed genotyping platform (Immunochip). Additional genetic variants and the classical HLA alleles were imputed and analyzed. The study identified and confirmed two independent susceptibility loci within the HLA region (r2 < 0.2): HLA-B/MICA (rs12524487, OR = 3.29, p = 5.57 × 10-16) and HLA-DQB1/HLA-DRB1 (rs113452171, OR = 2.34, p = 3.74 × 10-9; and rs189754752, OR = 2.47, p = 4.22 × 10-9). In addition, a genetic association was identified and confirmed between Takayasu's arteritis and the FCGR2A/FCGR3A locus on chromosome 1 (rs10919543, OR = 1.81, p = 5.89 × 10-12). The risk allele in this locus results in increased mRNA expression of FCGR2A. In addition, a genetic association between IL12B and Takayasu arteritis was established (rs56167332, OR = 1.54, p = 2.18 × 10-8). A fifth genetic locus for the disease on chromosome 21q22 downstream of PSMG1 was also revealed (P=4.39X10-7).

Diagnosis

Diagnosis is based on the demonstration of vascular lesions in large and middle-sized vessels on angiography, CT, or magnetic resonance angiography.

Contrast angiography has been the gold standard. The earliest detectable lesion is a local narrowing or irregularity of the lumen. This may develop into stenosis and occlusion. The characteristic finding is the presence of "skip lesions," where stenosis or aneurysms alterante with normal vessels. Angiography provides information on vessel anatomy and patency, but does not provide information on the degree of inflammation in the wall.

The age at onset helps to differentiate Takayasu's arteritis from other types of large vessel vasculitis such as. For example, Takaysu's arteritis has an age of onset of <40 years, while Giant Cell Arteritis has an age of onset >60 years.

Takayasu arteritis is not associated with ANCA, Rheumatoid factor, ANA, and Anticardiolipin antibodies.

Treatments

Most people with Takayasu’s arteritis respond to steroids such as prednisone. The usual starting dose is approximately 1 milligram per kilogram of body weight per day (for most people, this is approximately 60 milligrams a day). Because of the significant side effects of long-term high–dose prednisone use, the starting dose is tapered over several weeks to a dose that the physician feels is tolerable for the patient. Promising results are achieved with mycophenolate and tocilizumab. If treatment is not kept to a high standard then long term damage or death can occur. Stress is a major factor that should be avoided at all costs; if this is not taken into account the surge of adrenaline can have a damaging effect on the heart.

Surgical options may need to be explored for those who do not respond to steroids. Re-perfusion of tissue can be achieved by large vessel reconstructive surgery such as bypass grafting. Grafting autologous tissue has the highest rates of success. Stenting often obviates the need for surgery. Percutaneous transluminal coronary angioplasty (PTCA) is not as effective in the long term but has fewer risks.

History

The first case of Takayasu’s arteritis was described in 1908 by Japanese ophthalmologist Mikito Takayasu at the Annual Meeting of the Japan Ophthalmology Society. Takayasu described a peculiar "wreathlike" appearance of the blood vessels in the back of the eye (retina). Two Japanese physicians at the same meeting (Drs. Onishi and Kagoshima) also reported similar eye findings in individuals whose wrist pulses were absent. It is now known that the blood vessel malformations that occur in the retina are an angiogenic response to the arterial narrowings in the neck, and that the absence of pulses noted in some people occurs because of narrowings of the blood vessels to the arms. The eye findings described by Takayasu are rarely seen in patients from North America and British Columbia.

References

External links

• Johns Hopkins Vasculitis Center Discusses Takayasu's Arteritis
• 00389 at CHORUS

• -362086386 at GPnotebook

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 高安動脈炎の臨床的特徴および診断 clinical features and diagnosis of takayasu arteritis
• 2. 高安動脈炎の治療 treatment of takayasu arteritis
• 3. 成人における血管炎の概要およびアプローチ overview of and approach to the vasculitides in adults
• 4. 小児血管炎：分類および発生率 vasculitis in children classification and incidence
• 5. 鎖骨下動脈盗血症候群 subclavian steal syndrome

English Journal

• The heart in vasculitis.

• Miloslavsky E, Unizony S.Author information Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, USA.AbstractAny cardiac tissue can be affected in most primary vasculitides. However, certain entities such as eosinophilic granulomatosis with polyangiitis (or Churg-Strauss syndrome) and Takayasu arteritis involve the heart in up to 60% of patients. Vasculitic cardiac complications are important to recognize because they have been linked to increased mortality and impact the treatment decision-making process. This article reviews the spectrum of cardiac manifestations in adults with large-, medium-, and small-vessel primary systemic vasculitides as well as their diagnosis and treatment.
• Rheumatic diseases clinics of North America.Rheum Dis Clin North Am.2014 Feb;40(1):11-26. doi: 10.1016/j.rdc.2013.10.006.
• Any cardiac tissue can be affected in most primary vasculitides. However, certain entities such as eosinophilic granulomatosis with polyangiitis (or Churg-Strauss syndrome) and Takayasu arteritis involve the heart in up to 60% of patients. Vasculitic cardiac complications are important to recognize
• PMID 24268007

• Optimizing the outcome of vascular intervention for Takayasu arteritis.

• Perera AH, Youngstein T, Gibbs RG, Jackson JE, Wolfe JH, Mason JC.Author information Imperial Vascular Unit, St Mary's Hospital, London, UK.AbstractBACKGROUND: Takayasu arteritis (TA) predisposes to the development of arterial stenoses and aneurysms, and is associated with considerable morbidity and mortality amongst young patients. The aims of this study were to analyse indications and outcomes of surgical intervention, and to assess the potential benefits of immunosuppression and the use of perioperative imaging.
• The British journal of surgery.Br J Surg.2014 Jan;101(2):43-50. doi: 10.1002/bjs.9372.
• BACKGROUND: Takayasu arteritis (TA) predisposes to the development of arterial stenoses and aneurysms, and is associated with considerable morbidity and mortality amongst young patients. The aims of this study were to analyse indications and outcomes of surgical intervention, and to assess the poten
• PMID 24375298

• Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies.

• Clifford A, Hoffman GS.Author information Alison Clifford and Gary S Hoffman are both affiliated with the Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio, USA.AbstractPURPOSE OF REVIEW: To critically review recent advances in medical management of Takayasu arteritis, with a special focus on the rationale and evidence to support the use of biologic agents in this disease.
• Current opinion in rheumatology.Curr Opin Rheumatol.2014 Jan;26(1):7-15. doi: 10.1097/BOR.0000000000000004.
• PURPOSE OF REVIEW: To critically review recent advances in medical management of Takayasu arteritis, with a special focus on the rationale and evidence to support the use of biologic agents in this disease.RECENT FINDINGS: Multiple case series and observational studies support the use of anti-tumor
• PMID 24225487

Japanese Journal

• 高安動脈炎の診断と治療 : 生物学的製剤による治療の現状に焦点を当てて (第1土曜特集 血管炎の診断と治療 : 新分類CHCC2012に沿って) -- (大型血管炎)

• 中岡 良和
• 医学のあゆみ 246(1), 5-11, 2013-07-06
• NAID 40019681447

• 頸部痛と発熱で発症した早期高安動脈炎の1例

• 丸山 裕美子,北川 典子,伊藤 真人 [他]
• 日本耳鼻咽喉科学会会報 116(6), 709-714,図版1枚, 2013-06
• NAID 40019747092

• 医学と医療の最前線 高安動脈炎

• 磯部 光章
• 日本内科学会雑誌 102(4), 986-993, 2013-04-10
• NAID 40019674998

Related Links

• Takayasu's arteritis - Wikipedia, the free encyclopedia

Takayasu's disease (also known as "aortic arch syndrome", "nonspecific aortoarteritis" and the "pulseless disease" :841) is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, affecting often ...

• Takayasu's arteritis - MayoClinic.com

Takayasu's arteritis — Comprehensive overview covers symptoms, diagnosis, treatment of this rare blood vessel disease.

Related Pictures

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★リンクテーブル★

リンク元	「大動脈炎症候群」「血管炎」「TA」「Takayasu動脈炎」

「大動脈炎症候群」

　　[★]

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

• 中・大動脈を冒す炎症性の疾患で、血管腔の狭窄による症状を引き起こす。
• 大動脈瘤(aortic aneyrysm)や大動脈解離(aortic dissection)

• 特定疾患治療研究事業(特定疾患)

病因

• 不明

• ウイルス？、自己免疫的な機序
• HLAとの関連：HLA-B52、HLA-B39 (YN.C-151)

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)

• 顕微鏡的所見：

• 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

• 動脈硬化症、炎症性腹部大動脈瘤、血管型ベーチェット病、梅毒性中膜炎、巨細胞性動脈炎、先天性血管異常、細菌性動脈瘤 (参考1-1)
• 側頭動脈炎、結節性多発性動脈炎、川崎病など

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

• 糖質コルチコイド
• 免疫抑制薬

• 糖質コルチコイドでコントロールできない場合。

外科療法＆カテーテルインターベンション

• 血管内腔の狭窄に対して

外科療法

• バイパス術

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)

• 大動脈瘤破裂が増加傾向 (YN.C-151)

予後

• 5年生存率：80-90%(PHD.361)

参考

• 1. 大動脈炎症候群 - 難病情報センター

http://www.nanbyou.or.jp/entry/290

• 1-1. 認定基準

http://www.nanbyou.or.jp/upload_files/065_s.pdf

• 2. 血管炎症候群の診療ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf

• 3. まとまっている

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

「血管炎」

　　[★]

英

angiitis

関

脈管炎 vasculitis　vasculitides、血管内膜炎、血管炎症候群

分類

CHCC2012

large vessel vasculitis LVV 大型血管炎	Takayasu arteritis TAK 高安動脈炎
	giant cell arteritis GCA 巨細胞性動脈炎
medium vessel vasculitis MVV 中型血管炎	polyarteritis nodosa PAN 結節性多発動脈炎
	Kawasaki disease KD 川崎病
small vessel vasculitis SVV 小型血管炎	antineutrophil cytoplasmic antibody-associated vasculitis AAV 抗好中球細胞質抗体関連血管炎	microscopic polyangiitis MPA 顕微鏡的多発血管炎
		granulomatosis with polyangiitis GPA 多発血管炎性肉芽腫症(Wegener肉芽腫症)
		eosinophilic granulomatosis with polyangiitis EGPA 好酸球性多発血管炎性肉芽腫症(Churg-Strauss症候群)
	immune complex SVV 免疫複合体性小型血管炎	anti-glomerular basement membrane disease 抗糸球体基底膜抗体病(抗GBM病)
		cryoglobulinemic vasculitis CV クリオグロブリン血症性血管炎
		IgA vasculitis IgAV IgA血管炎(Henoch-Schonlein紫斑病)
		hypocomplementemic urticarial vasculitis HUV 低補体血症性蕁麻疹様血管炎(抗C1q血管炎)
variable vessel vasculitis VVV 多様な血管を侵す血管炎	Behcet's disease BD Behcet病
	Cogan's syndrome CS Cogan症候群
single-organ vasculitis SOV 単一臓器血管炎	cutaneous leukocytoclastic angiitis 皮膚白血球破砕性血管炎
	cutaneous arteritis 皮膚動脈炎
	primary central nervous system vasculitis 原発性中枢神経系血管炎
	isolated aortitis 限局性大動脈炎
vasculitis associated with systemic disease 全身性疾患関連血管炎	lupus vasculitis ループス血管炎
	rheumatoid vasculitis リウマトイド血管炎
	sarcoid vasculitis サルコイド血管炎
vasculitis associated with probable etiology 推定病因を有する血管炎	hepatitis C virus-associated cryoglobulinemic vasculitis C型肝炎ウイルス関連クリオグロブリン血症性血管炎
	hepatitis B virus-associated vasculitis B型肝炎ウイルス関連血管炎
	syphilis-associated aortitis 梅毒関連大動脈炎
	drug-associated immune complex vasculitis 薬剤関連免疫複合体性血管炎
	drug-associated ANCA-associated vasculitis 薬剤関連ANCA関連血管炎
	cancer-associated vasculitis がん関連血管炎

参考

• 血管炎症候群 - 日本内科学会雑誌第103巻第10号

• 1. 血管炎症候群の診療ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf

• 血管炎・血管障害診療ガイドライン 2016 年改訂版 - 日本皮膚科学会

https://www.dermatol.or.jp/uploads/uploads/files/vasculitisGL.pdf

「TA」

　　[★]

• 総動脈管 truncus artenosus
• 血小板無力症 thrombasthenia = グランツマン血小板無力症 = Glanzmann thrombasthenia
• 三尖弁閉鎖症 tricuspid atresia
• 高安動脈炎 Takayasu arteritis、高安病 Takayasu's disease Takayasu disease =大動脈炎症候群 aortitis syndrome、脈なし病

「Takayasu動脈炎」

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英

Takayasu arteritis

関

高安動脈炎