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| {{DiseaseDisorder infobox | | | {{Takayasu's arteritis}} |
| Name = {{PAGENAME}} |
| | {{CMG}}; {{AE}} {{FKH}}, {{SHH}} |
| ICD10 = {{ICD10|M|31|4|m|30}} |
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| ICD9 = {{ICD9|446.7}} |
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| ICDO = |
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| Image = Microscopic takayasu.jpg|
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| Caption = Takayasu's arteritis. <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
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| OMIM = 207600 |
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| MedlinePlus = 001250 |
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| eMedicineSubj = |
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| eMedicineTopic = |
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| eMedicine_mult = |
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| DiseasesDB = 12879 |
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| MeshID = D013625 |
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| }}
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| {{Search infobox}} | |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
| | {{SK}}Takayasu arteritis; pulseless disease; young female arteritis; young female arteritides; Takayasu syndrome; Takayasu disease; aortitis syndrome |
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| {{CMG}}'''; Associate Editor-In-Chief:''' {{CZ}}
| | ==[[Takayasu's arteritis overview|Overview]]== |
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| {{SK}} Aortic arch syndrome, nonspecific aortoarteritis, pulseless disease
| | ==[[Takayasu's arteritis historical perspective|Historical Perspective]]== |
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| ==Overview== | | ==[[Takayasu's arteritis classification|Classification]]== |
| Takayasu's arteritis is an [[inflammation|inflammatory disease]] of unknown [[etiology]] that affects mainly the [[aorta]] and its branches. Takayasu's arteritis is a form of large vessel [[granulomatous]] [[vasculitis]] characterized by massive [[intima]]l [[fibrosis]] and vascular narrowing. 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".
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| ==Historical Perspective== | | ==[[Takayasu's arteritis pathophysiology|Pathophysiology]]== |
| The first case of Takayasu’s arteritis was described in 1908 by Dr. [[Mikito Takayasu]] at the Annual Meeting of the Japan Ophthalmology Society.<ref>{{WhoNamedIt|synd|2722}}</ref><ref>M. Takayasu. A case with peculiar changes of the central retinal vessels. Acta Societatis ophthalmologicae Japonicae, Tokyo 1908, 12: 554.</ref> Dr. Takayasu described a peculiar "wreathlike" appearance of [[blood vessel]]s in the back of the eye ([[retina]]). Two Japanese colleagues at the same meeting (Dr. Onishi and Dr. Kagoshima) reported similar eye findings in patients whose [[Pulse|wrist pulse]]s were absent. It is now known that the blood vessel malformations that occur in the retina are a response ([[angiogenesis|new blood vessel growth]]) to arterial narrowings in the neck, and that the absence of pulses noted in some patients occur because of narrowings of blood vessels to the arms. The eye findings described by Takayasu are rarely seen in patients from North America.
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| ==Classification== | | ==[[Takayasu's arteritis causes|Causes]]== |
| Four types of late-phase Takayasu arteritis are described on the basis of the sites of involvement as follows:<ref>{{cite web |url=http://www.emedicine.com/radio/topic51.htm |title=eMedicine - Arteritis, Takayasu : Article by Robert L Cirillo, Jr, MD, MBA |accessdate=2007-07-19 |format= |work=}}</ref>
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| * Type I - Classic pulseless type that involves the brachiocephalic trunk, carotid arteries, and subclavian arteries
| | ==[[Takayasu's arteritis differential diagnosis|Differentiating Takayasu's arteritis from other Diseases]]== |
| * Type II - Combination of type I and III
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| * Type III - Atypical coarctation type that involves the thoracic and abdominal aortas distal to the arch and its major branches
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| * Type IV - Dilated type that involves extensive dilatation of the length of the aorta and its major branches
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| ==Pathophysiology== | | ==[[Takayasu's arteritis epidemiology and demographics|Epidemiology and Demographics]]== |
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| Although its [[etiology|cause]] is unknown, the condition is characterized by segmental and patchy [[granuloma]]tous [[inflammation]] of the aorta and its major derivative branches. This inflammation leads to arterial [[stenosis]], [[thrombosis]], and [[aneurysm]]s. 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).
| | ==[[Takayasu's arteritis risk factors|Risk Factors]]== |
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| 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.<ref name=SARUHAN2013>{{cite journal|last=Saruhan-Direskeneli|first=G|coauthors=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|title=Identification of Multiple Genetic Susceptibility Loci in Takayasu Arteritis|journal=American journal of human genetics|date=Jul 2, 2013|pmid=23830517|volume=93|issue=2|pages=298–305|doi=10.1016/j.ajhg.2013.05.026|pmc=3738826}}</ref> 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).<ref name="SARUHAN2013"/>
| | ==[[Takayasu's arteritis screening|Screening]]== |
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| ==Epidemiology and Demographics== | | ==[[Takayasu's arteritis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| ===Race===
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| Although Takayasu's arteritis has been reported worldwide, there is a predilection for young Asian women. In the Western world, [[atherosclerosis]] is a more frequent cause of obstruction of the aortic arch vessels than is Takayasu's arteritis.
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| ===Age=== | | ==Diagnosis== |
| The age of onset is typically between 15 and 30 years.
| | [[Takayasu's arteritis diagnostic study of choice|Diagnostic study of choice]] | [[Takayasu's arteritis history and symptoms|History and Symptoms]] | [[Takayasu's arteritis physical examination|Physical Examination]] | [[Takayasu's arteritis laboratory findings|Laboratory Findings]] | [[Takayasu's arteritis electrocardiogram|Electrocardiogram]] | [[Takayasu's arteritis x ray|X-Ray]] | [[Takayasu's arteritis echocardiograhy and ultrasound|Echocardiography and Ultrasound]] | [[Takayasu's arteritis CT|CT Scan]] | [[Takayasu's arteritis MRI|MRI Findings]] | [[Takayasu's arteritis other imaging findings|Other Imaging Findings]] | [[Takayasu's arteritis other diagnostic studies|Other Diagnostic Studies]] |
| ===Gender===
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| Females are about 8–9 times more likely to be affected by Takayasu's arteritis than males.
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| ==Natural History, Complications and Prognosis== | | ==[[Treatment]]== |
| ===Natural History===
| | [[Takayasu's arteritis medical therapy|Medical Therapy]] | [[Takayasu's arteritis surgery|Surgery]] | [[Takayasu's arteritis primary prevention|Primary Prevention]] | [[Takayasu's arteritis secondary prevention|Secondary Prevention]] | [[Takayasu's arteritis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Takayasu's arteritis future or investigational therapies|Future or Investigational Therapies]] |
| Those with the disease often notice symptoms between 15 and 30 years of age. Some people develop an initial "inflammatory phase" characterized by systemic illness with signs and [[symptom]]s of [[malaise]], [[fever]], [[sleep hyperhidrosis|night sweats]], [[weight loss]], [[arthralgia|joint pain]], [[fatigue (physical)|fatigue]], and [[Syncope (medicine)|fainting]]. Fainting may result from [[subclavian steal syndrome]] or [[carotid sinus]] hypersensitivity.<ref>Shikino Kiyoshi, Takako Masuyama and Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014.</ref> There is also often [[anemia]] and marked elevation of the [[Erythrocyte sedimentation rate|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 [[human eye|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 [[aneurysm]]s. 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.
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| ===Complications===
| | [[Category:Medicine]] |
| Cardiac complications of [[takayasu arteritis]] may include [[aortic regurgitation]], [[myocarditis]] and [[congestive heart failure]].
| | [[Category:Rheumatology]] |
| | | [[Category:Up-To-Date]] |
| ===Prognosis===
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| Five year survival in TA is over 90%, but TA is associated with significant morbidity.
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| ==Symptoms==
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| The disease can be divided into two phases; the Initial phase is pre-pulseless phase, in which patients presents which non-specific constitutional symptoms of [[vasculitis]], which may include any of the following:
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| * [[Fatigue]]
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| * [[Fever of unknown origin]]
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| * [[Weight loss]]
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| * [[Myalgia]]
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| * [[Arthralgia]]
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| With progression of the disease and involvement of the branches of aorta,<ref name="pmid7909656">{{cite journal| author=Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M et al.| title=Takayasu arteritis. | journal=Ann Intern Med | year= 1994 | volume= 120 | issue= 11 | pages= 919-29 | pmid=7909656 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7909656 }} </ref> the specific signs appear secondary to narrowing/occlusion of the branches of aorta.
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| * Involvement of [[subclavian artery]] is common and leads to claudication of upper extremities (pain with activity). The stenosis of subclavian artery sometimes leads to [[subclavian steel syndrome]]<ref name="pmid15335">{{cite journal| author=Yoneda S, Nukada T, Tada K, Imaizumi M, Takano T| title=Subclavian steal in Takayasu's arteritis. A hemodynamic study by means of ultrasonic Doppler flowmetry. | journal=Stroke | year= 1977 | volume= 8 | issue= 2 | pages= 264-8 | pmid=15335 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15335 }} </ref>. In this phenomenon, the stenosis of subclavian artery, proximal to the origin of Vertebral Artery leads to retrograde flow of blood from the vertebral artery back to subclavian artery during exercise, secondary to vasodilation of blood vessels. The retrograde flow of blood from the vertebral artery back towards subclavian compromises blood flow in [[posterior cerebral bed]], leading to various neurological symptoms including presyncope/syncope.
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| * Involvement of carotid and vertebral arteries: headache, vertigo, syncope, convulsions and dementia
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| * Involvement of coronary arteries: chest pain, angina which may progress to myocardial infarction
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| * Involvement of ascending aorta may: aortic regurgiatation
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| * Skin lesions resemble erythema nodosum, erythema multiforme, pyoderma gangrenosum
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| In rare instances, the disease may involve abdominal, pulmonary vessels. In advance stages of the disease, the occlusion of the vessels to the extremities may can ischemic ulcerations. Due to chronic nature of the disease, collateral circulation develops in the affected area.
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| ==Diagnosis==
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| Initially, a diagnosis of TA can be missed for months or years because its symptoms can be nonspecific ([[fever]], [[lethargy]], etc.). The American College of Rheumatology (ACR) has established diagnostic criteria for Takayasu arteritis. The patient needs to meet 3 out of 6 criteria for the diagnosis of Takayasu's arteritis:<ref name="pmid1975175">Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM et al. (1990) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1975175 The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis.] ''Arthritis Rheum'' 33 (8):1129-34. PMID: [http://pubmed.gov/PMID: 1975175 PMID: 1975175]</ref>
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| * Age at the onset of disease <40 years
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| * [[Claudication]] of the extremities
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| * Decreased or absent [[brachial artery]] pulse in one or both arms
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| * Difference of at least 10 mmHg in [[systolic blood pressure]] between the arms
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| * Bruit over either one or both [[subclavian arteries]] or abdominal artery
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| * Evidence of narrowing of aorta or its primary branches on [[arteriography]], not due to atherosclerosis, fibromuscular dyaplasia, or other causes
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| ===Physical Examination===
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| While Takayasu arteritis is sometimes referred to as the "pulseless disease", it is important to note that a missing peripheral pulse usually occurs late in the course of TA. More often individuals with TA present with an asymmetric pulse.<ref name="urlTakayasu Arteritis: eMedicine Pediatrics: General Medicine">{{cite web |url=http://emedicine.medscape.com/article/1007566-overview |title=Takayasu Arteritis: eMedicine Pediatrics: General Medicine |format= |work= |accessdate=}}</ref>
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| ===Imaging Studies===
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| The diagnosis of TA is sometimes made when a [[widened mediastinum]] is noted on a [[chest x ray]]. A follow-up CT scan will often demonstrate a widened aortic arch. <ref name="urlTakayasu Arteritis: eMedicine Pediatrics: General Medicine">{{cite web |url=http://emedicine.medscape.com/article/1007566-overview |title=Takayasu Arteritis: eMedicine Pediatrics: General Medicine |format= |work= |accessdate=}}</ref>
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| ====Computed Tomography and Magnetic Resonance Angiography====
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| Computed Tomography (CT) and Magnetic Resonance Angiography (MRA) are the gold standrad imaging modalities for TA and may demonstrate mural thickening of the aorta and luminal narrowing. Use of contrast may reveal inflammatory lesions prior to the development of stenoses; these lesions may be missed by angiography. Aortic lesions including stenosis, dilatation, wall thickening, and mural thrombi are well visualized on MRI, which is less adequate in visualizing distal lesions of the subclavian vessels and common carotids. <ref>Yamada I, Nakagawa T, Himeno Y: Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three- dimensional MR angiography. J Magn Reson Imaging 2000; 11: 481</ref> Non-contrast T2-weighted STIR images may be used to monitor edema in the aortic wall, which may be a surrogate for inflammation; edema may present more than 94% of patients with clinically active disease. One disadvantage of MRA is that pressure differentials cannot be measured across lesions in which imaging findings regarding their hemodynamic significance are inconclusive.
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| <div align="center">
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| <gallery heights="275" widths="270">
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| Image:Takayasu.jpg|This MR angiogram demonstrates findings typical of Takayasu arteritis. A 32 years old Asian female has CRF and weak functioning iatrogenic fistula in left upper limb. The cause of malfunction can be explained by narrowing and occlusion of upper limb arteries. Notice also narrowed abdominal aorta. (Image courtesy of Dr Ahmed Haroun)
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| Image:Takayasu_arteritis.JPG|MRA: A 15 year-old girl with known Takayasu arteritis presented for MRI to investigate back pain. The axial T1-weighted post-gadolinium MRI above shows thickened, enhancing aortic wall, consistent with large vessel vasculitis. (Image courtesy of Dr Laughlin Dawes)
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| </gallery>
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| </div>
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| '''Warning:''' Gadolinium-based contrast agents [gadopentetate dimeglumine (Magnevist), gadobenate dimeglumine (MultiHance), gadodiamide (Omniscan), gadoversetamide (OptiMARK), gadoteridol (ProHance)] have recently been linked to the development of [[nephrogenic systemic fibrosis]] (NSF) or [[nephrogenic fibrosing dermopathy]] (NFD). The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. In December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. <ref>www.fda.gov</ref>
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| While computed tomography and MRA are the best imaging modalities, findings on other imaging modalities include:
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| ====Chest radiography====
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| Chest radiographs may reveal widening of the ascending aorta, irregular descending aorta, aortic calcifications, and rib notching (late findings).
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| ====Duplex Doppler====
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| This modality may be used to evaluate and monitor disease in the common carotids and subclavian arteries; however, this imaging study is not useful in evaluating the aorta.
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| ====Carotid Ultrasound====
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| Carotid ultrasound demonstrates a homogenous circumferential thickening of the vessel wall that is distinguishable from atherosclerotic thickening.
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| ====Gallium-67 radionuclide scan====
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| This scan may demonstrate increased uptake in the aorta and branches.
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| ====MRI====
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| Shown below is an MRI of a 15 year-old female patient with known Takayasu arteritis, showing thickened, enhancing aortic wall, consistent with large vessel vasculitis.
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| [[File:Takayasu arteritis.JPG|300px]]
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| ====Angiography====
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| ==Treatment==
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| ===Medical Therapy===
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| 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 [[Adverse effect (medicine)|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.
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| 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.
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| 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.
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| ===Surgery===
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| 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.
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| Percutaneous transluminal coronary angioplasty (PTCA) is not as effective in the long term but has fewer risks.
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| ==References==
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| {{Reflist|2}}
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| {{Diseases of the musculoskeletal system and connective tissue}}
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| [[Category:Angiology]]
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| [[Category:Cardiovascular diseases]]
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| [[Category:Cardiology]]
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| [[Category:Disease]]
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