WordNet

an impairment of health or a condition of abnormal functioning
caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

(体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
女性の話術芸人 =diseur
病気にかかった / 病的な,不健全な(morbid)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/02/07 15:25:36」(JST)

wiki en

Takayasu's disease (also known as "aortic arch syndrome", "nonspecific aortoarteritis" and the "pulseless disease"^[1]^:841) is a form of large vessel granulomatous vasculitis^[2] 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.^[2]^[3] 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.^[2] 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".

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.^[4] 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".^[2] 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.^[2] 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.^[5]^[6] 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. Raynaud's phenomenon is commonly found in this disease, mainly due to decreased circulation of the blood to the arms.

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.^[3] 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).^[5] 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.^[7] 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).^[7]

Diagnosis

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.^[8]^[9] 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^{[citation needed]}.

References

^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
^ ^a ^b ^c ^d ^e American College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology. "Systemic Vasculitis." Pg. 65–67. 2009, ACP. [1]
^ ^a ^b Takayasu Arteritis - Pediatrics at eMedicine
^ Shikino Kiyoshi, Takako Masuyama and Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014.
^ ^a ^b John Barone, M.D. USMLE Step 1 Lecture Notes. "Vascular Pathology." 2008, Kaplan Inc. pg 101.
^ Milan B, Josip K. (November 1967). "Ocular manifestations of the aortic arch syndrome (pulseless disease; Takayasu's disease) (Translated from French)". Annales d'oculistique 200 (11): 1168–79. PMID 6079381.
^ ^a ^b Saruhan-Direskeneli, G; Hughes, T; Aksu, K; Keser, G; Coit, P; Aydin, SZ; Alibaz-Oner, F; Kamalı, S; Inanc, M; Carette, S; Hoffman, GS; Akar, S; Onen, F; Akkoc, N; Khalidi, NA; Koening, C; Karadag, O; Kiraz, S; Langford, CA; McAlear, CA; Ozbalkan, Z; Ates, A; Karaaslan, Y; Maksimowicz-McKinnon, K; Monach, PA; Ozer, HT; Seyahi, E; Fresko, I; Cefle, A; Seo, P; Warrington, KJ; Ozturk, MA; Ytterberg, SR; Cobankara, V; Onat, AM; Guthridge, JM; James, JA; Tunc, E; Duzgun, N; Bıcakcıgil, M; Yentür, SP; Merkel, PA; Direskeneli, H; Sawalha, AH (Jul 2, 2013). "Identification of Multiple Genetic Susceptibility Loci in Takayasu Arteritis". American journal of human genetics 93 (2): 298–305. doi:10.1016/j.ajhg.2013.05.026. PMC 3738826. PMID 23830517. Cite uses deprecated parameters (help)
^ synd/2722 at Who Named It?
^ M. Takayasu. A case with peculiar changes of the central retinal vessels. Acta Societatis ophthalmologicae Japonicae, Tokyo 1908, 12: 554.

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. Overview of and approach to the vasculitides in adults
4. Approach to the infant or child with a cardiac murmur
5. 小児血管炎：分類および発生率 vasculitis in children classification and incidence

English Journal

Outcome and incidence of appropriate implantable cardioverter-defibrillator therapy in patients with cardiac amyloidosis.

Hamon D1, Algalarrondo V2, Gandjbakhch E3, Extramiana F4, Marijon E5, Elbaz N6, Selhane D2, Dubois-Rande JL6, Teiger E6, Plante-Bordeneuve V7, Damy T8, Lellouche N8.
International journal of cardiology.Int J Cardiol.2016 Nov 1;222:562-8. doi: 10.1016/j.ijcard.2016.07.254. Epub 2016 Aug 1.
BACKGROUND: Cardiac amyloidosis (CA) is associated with a poor prognosis with the proposed mechanism of sudden cardiac death in the majority of patients being pulseless electrical activity. However, the incidence of ventricular arrhythmias (VA) and implantable cardioverter-defibrillator (ICD) indica
PMID 27513652

Management of a Thoracic Aortic Aneurysm during Pregnancy Leading to the Diagnosis of Takayasu Arteritis.

Jérémie J1, Julien G2, Marc D2, Nicolas K2, Miguel H2, Ismail K2, Laurent C2, Fabien K2.
Annals of vascular surgery.Ann Vasc Surg.2016 Oct;36:291.e1-291.e4. doi: 10.1016/j.avsg.2016.02.040. Epub 2016 Jul 6.
Takayasu arteritis is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. We report the case of a patient presenting with the rapid growth of a thoracic aortic aneurysm during pregnancy leading to the diagnosis of Takayasu disease and treated by a
PMID 27395815

Large vessel vasculitis: the search for response biomarkers.

Tombetti E1, Mason J2.
Expert review of clinical immunology.Expert Rev Clin Immunol.2016 Oct;12(10):1011-3. doi: 10.1080/1744666X.2016.1224180. Epub 2016 Aug 23.
PMID 27551980

Japanese Journal

経皮的心肺補助法(PCPS)合併症を含めた，全身管理に苦慮した劇症型心筋炎の1例

田川実,高橋一也,中村裕一,渡邉雅貴,落合幸江,高田琢磨,目黒昌,池主雅臣,小玉誠,相澤義房
Shinzo 45(8), 1016-1023, 2013
… 状を認めたが，2010年2月呼吸困難が出現し，当院救急外来を受診した．X線写真で心拡大と両側胸水を認め，心臓超音波検査(ultrasonic echocardiography；UCG)で心機能低下を認めた．当院到着直後に無脈性電気活動(pulseless electrical activity；PEA)となり，心肺蘇生を行いながら，大動脈バルーンパンピング(intra aortic balloon pumping；IABP)を挿入した．冠動脈造影で有意狭窄はなく，左室造影で著明な壁運動低下を認めた．劇症型心筋炎 …
NAID 130004691774

胸骨圧迫に起因すると考えられる肝損傷から出血性ショックに至った１例

鈴木裕之,中野実,蓮池俊和,仲村佳彦,畠山淳司,庭前野菊,清水尚
日本救急医学会雑誌 22(6), 297-302, 2011
… 救急車内収容時に無脈性電気活動（pulseless electrical activity; …
NAID 130004542320

Atypical Giant Cell Arteritis Presenting as Lack of a Pulse in the Upper Extremity

Shibutani Shintaro,Obara Hideaki,Kan Shigenao,Takahashi Shin,Ogawa Kenji,Kitagawa Yuko
Annals of Vascular Diseases 4(3), 241-244, 2011
… GCA should be considered along with atherosclerosis in cases of occlusive disease of the upper extremity, especially if the patient is an elderly woman. …
NAID 130004487917

Related Pictures

Takayasu's arteritis (pulseless disease) | Radiology Case | Radiopaedia.org Takayasu's arteritis - Wikipedia

★リンクテーブル★

リンク元	「大動脈炎症候群」
関連記事	「disease」

「大動脈炎症候群」

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

　　[★]

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

「disease」

　　[★]

n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

something that is very wrong with people's attitudes, way of life or with society.

関: ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

注意

disease ≠ illness ≠ disorder

[Andrews-1] James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.

[mk-2] American College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology. "Systemic Vasculitis." Pg. 65–67. 2009, ACP. [1]

[emedpedtakay-3] Takayasu Arteritis - Pediatrics at eMedicine

[4] Shikino Kiyoshi, Takako Masuyama and Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014.

[kaplan-5] John Barone, M.D. USMLE Step 1 Lecture Notes. "Vascular Pathology." 2008, Kaplan Inc. pg 101.

[6] Milan B, Josip K. (November 1967). "Ocular manifestations of the aortic arch syndrome (pulseless disease; Takayasu's disease) (Translated from French)". Annales d'oculistique 200 (11): 1168–79. PMID 6079381.

[SARUHAN2013-7] Saruhan-Direskeneli, G; Hughes, T; Aksu, K; Keser, G; Coit, P; Aydin, SZ; Alibaz-Oner, F; Kamalı, S; Inanc, M; Carette, S; Hoffman, GS; Akar, S; Onen, F; Akkoc, N; Khalidi, NA; Koening, C; Karadag, O; Kiraz, S; Langford, CA; McAlear, CA; Ozbalkan, Z; Ates, A; Karaaslan, Y; Maksimowicz-McKinnon, K; Monach, PA; Ozer, HT; Seyahi, E; Fresko, I; Cefle, A; Seo, P; Warrington, KJ; Ozturk, MA; Ytterberg, SR; Cobankara, V; Onat, AM; Guthridge, JM; James, JA; Tunc, E; Duzgun, N; Bıcakcıgil, M; Yentür, SP; Merkel, PA; Direskeneli, H; Sawalha, AH (Jul 2, 2013). "Identification of Multiple Genetic Susceptibility Loci in Takayasu Arteritis". American journal of human genetics 93 (2): 298–305. doi:10.1016/j.ajhg.2013.05.026. PMC 3738826. PMID 23830517. Cite uses deprecated parameters (help)

[8] synd/2722 at Who Named It?

[9] M. Takayasu. A case with peculiar changes of the central retinal vessels. Acta Societatis ophthalmologicae Japonicae, Tokyo 1908, 12: 554.

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pulseless disease