World Library  
Flag as Inappropriate
Email this Article

Takayasu's arteritis

Article Id: WHEBN0001255502
Reproduction Date:

Title: Takayasu's arteritis  
Author: World Heritage Encyclopedia
Language: English
Subject: List of MeSH codes (C14), Vasculitis, Relapsing polychondritis, ICD-10 Chapter XIII: Diseases of the musculoskeletal system and connective tissue, Takayasu
Publisher: World Heritage Encyclopedia

Takayasu's arteritis

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-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"[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 which typically affects older individuals.[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". Involvement of renal arteries may lead to presentation as renovascular hypertension.


  • Sign and symptoms 1
  • Pathophysiology 2
  • Diagnosis 3
  • Treatments 4
  • History 5
  • References 6
  • External links 7

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. In view of chronic process and good collateral development, Raynaud's phenomenon or digital gangrene are very rare in Takayasu arteritis.


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 is based on the demonstration of vascular lesions in large and middle-sized vessels on angiography, CT, or magnetic resonance angiography. [8]

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. [8]

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. [8]

Takayasu arteritis is not associated with ANCA, Rheumatoid factor, ANA, and Anticardiolipin antibodies. [8]


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.


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.[9][10] 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.


  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier.  
  2. ^ 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. [2]
  3. ^ a b Takayasu Arteritis - Pediatrics at eMedicine
  4. ^ Shikino Kiyoshi, Takako Masuyama and Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014.
  5. ^ a b 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.  
  7. ^ 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.  
  8. ^ a b c d RA Watts et al., "Vasculitis in Clinical Medicine, 2010"
  9. ^ synd/2722 at Who Named It?
  10. ^ 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
This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from Project Gutenberg are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.