You are in: eMedicine Specialties > Radiology > VASCULAR/INTERVENTIONAL Arteritis, TakayasuArticle Last Updated: Sep 10, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Robert L Cirillo Jr, MD, MBA, Assistant Professor of Radiology, Florida State University College of Medicine; Medical Interventional Radiologist, Director/CEO, South Georgia Vascular Institute and South Georgia Laser Vein Center Robert L Cirillo, Jr, is a member of the following medical societies: American College of Physician Executives, Cardiovascular and Interventional Radiological Society of Europe, Society for Vascular Technology, and Society of Interventional Radiology Editors: Anthony Watkinson, MD, Professor of Interventional Radiology, The Peninsula Medical School; Consultant and Senior Lecturer, Department of Radiology, The Royal Devon and Exeter Hospital, UK; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Douglas M Coldwell, MD, PhD, Professor and Chief of Interventional Radiology, Professor of Radiology and Surgery, University of Missouri at Columbia; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Kyung J Cho, MD, FACR, William Martel Professor of Radiology, Interventional Radiology Fellowship Director, University of Michigan Health System Author and Editor Disclosure Synonyms and related keywords: Takayasu arteritis, Takayasu's arteritis, Takayasu disease, Takayasu's disease, nonspecific aortoarteritis, pulseless disease, aortic arch syndrome, granulomatous vasculitis, aortic inflammation INTRODUCTIONSection 2 of 11
  BackgroundTakayasu arteritis is a granulomatous vasculitis of unknown etiology that commonly affects the thoracic and abdominal aorta. It causes intimal fibroproliferation of the aorta, great vessels, pulmonary arteries, and renal arteries and results in segmental stenosis, occlusion, dilatation, and aneurysmal formation in these vessels. Takayasu arteritis is the only form of aortitis that causes stenosis and occlusion of the aorta.1, 2, 3, 4, 5, 6, 7 PathophysiologyThe mechanism of Takayasu disease has not been fully elucidated to date. It begins during the patient's first 2 decades of life as a nonspecific, cell-mediated inflammatory process; as the disease progresses, fibrotic stenoses form on the aorta and its major branches. FrequencyUnited StatesTakayasu arteritis afflicts approximately 1 person per 1000 in the United States. InternationalMost cases of Takayasu arteritis are reported in Asia, although the disease occurs worldwide. Takayasu arteritis afflicts 6 persons per 1000 worldwide. Mortality/MorbidityMortality related to Takayasu arteritis is usually the result of vascular complications such as hypertension, stroke, and aortic insufficiency. RaceClassic Takayasu arteritis is described in the Asian population; it is reported in all ethnic groups. SexTakayasu arteritis most commonly occurs in female patients; the female-to-male ratio is 8:1. AgeNinety percent of patients with Takayasu arteritis are younger than 30 years.4, 8, 9 Clinical DetailsTakayasu arteritis has early and late phases. The early phase is inflammatory and has been called the prepulseless phase; the late phase, called the pulseless phase, is characterized by occlusion. Patients may present with nonspecific signs and symptoms such as fever, arthralgias, and weight loss. During the acute inflammatory stage, Takayasu disease causes a low-grade fever, tachycardia, and pain adjacent to the inflamed arteries (eg, carotodynia)10; in addition, 50% of patients experience fatigability. Carotid and clavicular bruits, asymmetric upper-extremity blood pressures, hypertension, diminished or absent upper-extremity pulses, and ischemic symptoms may suggest the diagnosis. A 5- to 20-year interval may separate the acute inflammatory stage and the symptomatic arterial occlusive stage. Neurologic symptoms are present in 80% of patients with Takayasu disease that involves the brachiocephalic arteries. Four types of late-phase Takayasu arteritis have been described on the basis of the sites of involvement; those types are as follows:
The most common type is type III, which is found in as many as 65% of patients. The most commonly involved vessels include the left subclavian artery (50%), the left common carotid artery (20%), the brachiocephalic trunk, the renal arteries, the celiac trunk, the superior mesenteric artery, and the pulmonary arteries (50%). Infrequently, the axillary, brachial, vertebral, coronary, and iliac arteries are involved. Preferred ExaminationHistorically, angiography has been the criterion-standard imaging tool for the diagnosis and evaluation of Takayasu arteritis. In the past 5 years, computed tomography angiography (CTA) and magnetic resonance angiography (MRA) have become equally valuable tools. Their large fields of view, as well as the fact that they are noninvasive and that intravenously administered contrast material is used, rather than intra-arterially administered contrast material, make them far more attractive as diagnostic tools. The increasing resolution of multidetector-row CT arrays increases its diagnostic value. The soft tissue differentiation possible with MR techniques is valuable in distinguishing active forms of Takayasu disease from quiescent forms.11, 12, 13, 14 DIFFERENTIALSSection 3 of 11
Temporal Arteritis Other Problems To Be ConsideredAtherosclerosis: Atherosclerosis usually occurs in persons older than 40 years. It is more common in men than in women. It has a predilection for the origin of vessels and bifurcations in the neck Fibromuscular dysplasia (FMD): Classic FMD has a beaded appearance. It usually does not affect the aorta, as evidenced on angiograms. FMD is rare in the subclavian artery. Temporal arteritis: This disease usually does not involve the common carotid arteries. Syphilitic aortitis: Classically, syphilitic aortitis causes calcification in the ascending aorta. Neurofibromatosis type I, Williams syndrome, rubella, and radiation therapy may cause middle aortic syndrome; their manifestations may mimic Takayasu disease in this region. CT SCANSection 4 of 11
FindingsContrast-enhanced CTA may demonstrate thickening of the arterial wall with crescents and indistinct outlines. Multidetector-row CT now allows faster scanning at higher resolutions with large fields of view; these factors increase the diagnostic value of the tool. The utility of this noninvasive technique is particularly high in pediatric patients, in whom the complications of angiography are potentially worse than they are in adults.15, 16 One disadvantage of CTA is that pressure differentials cannot be measured across lesions in which imaging findings regarding their hemodynamic significance are inconclusive. MRISection 5 of 11
FindingsIn cases of Takayasu arteritis, gadolinium-enhanced MRA may demonstrate thickening of the arterial wall with crescents and indistinct outlines. The utility of this noninvasive technique is particularly high in pediatric patients in whom the complications of angiography are potentially greater than in adults.17, 18, 19, 14, 20, 21 The soft tissue differentiation obtained with MR techniques may help distinguish the active or acute phase from the chronic phase of the disease. This capability may be important in the timing of catheter-based or other interventions. One disadvantage of MRA is that pressure differentials cannot be measured across lesions in which imaging findings regarding their hemodynamic significance are inconclusive. Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. ULTRASOUNDSection 6 of 11
FindingsSonography may depict vascular stenoses in accessible areas; their location may suggest the diagnosis of Takayasu arteritis. Degree of ConfidenceThe negative predictive value of a sonographic finding in establishing or excluding the diagnosis of Takayasu disease is insufficient. ANGIOGRAPHYSection 7 of 11
FindingsAngiography is a useful imaging modality in the detection of Takayasu arteritis. Because of its invasiveness, angiography is not the first-line study in most patients, particularly pediatric patients. Gadolinium-enhanced MRA findings may be diagnostic or strongly suggestive of the disease (see Images 1-3).22 Aortography reveals focal, smooth, symmetric narrowing of the aorta and multiple branch-vessel stenosis or occlusion. Stenosis is the most common finding, although arterial dilatation and aneurysms are often found. When Takayasu disease involves the subclavian artery, the lesion is a smoothly tapered stenosis; it begins within a few centimeters of the arch and extends to the origin of the vertebral artery. The lesions are often multiple and symmetric. Serial angiography is helpful in the initial diagnosis and follow-up of patients with Takayasu arteritis. Pressure measurements should be obtained in the ascending aorta and compared with measurements in the extremities. In 75% of patients, the sites of vascular involvement include the aortic arch and its branches. The most commonly involved aortic branches are the left subclavian artery, which is affected in 55% of patients, followed by the right subclavian artery (38%), the left common carotid artery (30%), and the right common carotid artery (15%). INTERVENTIONSection 8 of 11
Early corticosteroid therapy may lead to improvements in the clinical findings and may aid in subsidence of the active inflammatory process of Takayasu arteritis. Prednisone is usually effective in controlling the clinical symptoms of Takayasu arteritis and in controlling and decreasing the progression of active disease.7 ACKNOWLEDGMENTSSection 9 of 11
The author thanks his wife, Florence, for her support in allowing the time to both pursue academic endeavors and complete this project. MULTIMEDIASection 10 of 11
REFERENCESSection 11 of 11
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