eMedicine Specialties > Radiology > Vascular/Interventional

Arteritis, Takayasu

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
Contributor Information and Disclosures

Updated: Sep 10, 2008

Introduction

Background

Takayasu 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

Pathophysiology

The 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.

Frequency

United States

Prevalence of Takayasu arteritis is 2.6-6.4 persons per 1,000,000 population. The wide variation is attributed to genetic factors and difficulties in making an accurate diagnosis.

International

Worldwide incidence is estimated to be 2.6 cases per 1,000,000 population/y.

Mortality/Morbidity

Mortality related to Takayasu arteritis is usually the result of vascular complications such as hypertension, stroke, and aortic insufficiency.

Race

Classic Takayasu arteritis is described in the Asian population; it is reported in all ethnic groups.

Sex

Takayasu arteritis most commonly occurs in female patients; the female-to-male ratio is 8:1.

Age

Ninety percent of patients with Takayasu arteritis are younger than 30 years.4,8,9

Presentation

Takayasu 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:

  • Type I — Classic pulseless type that involves the brachiocephalic trunk, carotid arteries, and subclavian arteries
  • Type II — Combination of types I and III
  • Type III — Atypical coarctation type that involves the thoracic and abdominal aortas distal to the arch and its major branches
  • Type IV — Dilated type that involves extensive dilatation of the length of the aorta and its major branches

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 Examination

Historically, 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

Differential Diagnoses

Temporal Arteritis

Other Problems to Be Considered

Atherosclerosis: 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.

More on Arteritis, Takayasu

Overview: Arteritis, Takayasu
Imaging: Arteritis, Takayasu
Follow-up: Arteritis, Takayasu
Multimedia: Arteritis, Takayasu
References
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References

  1. Giordano JM. Takayasu''s disease and temporal arteritis. Semin Vasc Surg. 1995;8:335-41. [Medline].

  2. Jejeda C, Correa P. Non-syphilitic aortitis. Arch Pathology. 1969;87:177.

  3. Numano F, Okawara M, Inomata H. Takayasu''s arteritis. Lancet. Sep 16 2000;356:1023-5. [Medline].

  4. Cakar N, Yalcinkaya F, Duzova A, Caliskan S, Sirin A, Oner A, et al. Takayasu arteritis in children. J Rheumatol. May 2008;35(5):913-9. [Medline].

  5. Chung JW, Kim HC, Choi YH, Kim SJ, Lee W, Park JH. Patterns of aortic involvement in Takayasu arteritis and its clinical implications: evaluation with spiral computed tomography angiography. J Vasc Surg. May 2007;45(5):906-14. [Medline].

  6. Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis?. Rheum Dis Clin North Am. Nov 2007;33(4):777-86, vi. [Medline].

  7. Mwipatayi BP, Jeffery PC, Beningfield SJ, Matley PJ, Naidoo NG, Kalla AA, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg. Mar 2005;75(3):110-7. [Medline].

  8. Sharma S, Thataie D, Saxena A. Renovascular hypertension resulting from nonspecific aortoarteritis in children: midterm results of percutaneous transluminal renal angioplasty and predictors of restenosis. AJR Am J Roentgenol. 1996;166:157-162. [Medline].

  9. Ozen S, Duzova A, Bakkaloglu A, Bilginer Y, Cil BE, Demircin M, et al. Takayasu arteritis in children: preliminary experience with cyclophosphamide induction and corticosteroids followed by methotrexate. J Pediatr. Jan 2007;150(1):72-6. [Medline].

  10. Zeina AR, Slobodin G, Barmeir E. Takayasu's arteritis as a cause of carotidynia: clinical and imaging features. Isr Med Assoc J. Feb 2008;10(2):158-9. [Medline].

  11. Cantu C, Pineda C, Barinagarrementeria F. Noninvasive cerebrovascular assessment of Takayasu arteritis. Stroke. 2000;31:2197-2202. [Medline].

  12. Matsunaga N, Hayashi K, Sakamoto I. Takayasu arteritis: protean radiologic manifestations and diagnosis. Radiographics. May-Jun 1997;17(3):579-94. [Medline].

  13. Pipitone N, Versari A, Salvarani C. Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford). Apr 2008;47(4):403-8. [Medline].

  14. Yamada I, Numano F, Suzuki S. Takayasu arteritis: evaluation with MR imaging. Radiology. Jul 1993;188(1):89-94. [Medline].

  15. Paul JF, Fiessinger JN, Sapoval M. Follow-up electron beam CT for the management of early phase Takayasu arteritis. J Comput Assist Tomogr. 2001;25:924-31. [Medline].

  16. Gupta R, Kavimandan A, Kumar R. Does PET-CT predict disease activity in Takayasu's arteritis?. Scand J Rheumatol. May-Jun 2008;37(3):237-9. [Medline].

  17. Choe YH, Han BK, Koh EM. Takayasu''s arteritis: assessment of disease activity with contrast- enhanced MR imaging. AJR Am J Roentgenol. 2000;175:505-11. [Medline].

  18. Flamm SD, White RD, Hoffman GS. The clinical application of ''edema-weighted'' magnetic resonance imaging in the assessment of Takayasu''s arteritis. Int J Cardiol. Oct 1 1998;66 (suppl 1):S151-9; discussion S161. [Medline].

  19. Kumar S, Radhakrishnan S, Phadke RV. Takayasu''s arteritis: evaluation with three-dimensional time-of-flight MR angiography. Eur Radiol. 1997;7(1):44-50. [Medline].

  20. Yamada I, Nakagawa T, Himeno Y. Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three- dimensional MR angiography. J Magn Reson Imaging. 2000;11:481-7. [Medline].

  21. Steeds RP, Mohiaddin R. Takayasu arteritis: role of cardiovascular magnetic imaging. Int J Cardiol. Apr 28 2006;109(1):1-6. [Medline].

  22. Park JH, Han MC, Kim SH. Takayasu arteritis: angiographic findings and results of angioplasty. AJR Am J Roentgenol. Nov 1989;153(5):1069-74. [Medline].

  23. Liang P, Hoffman GS. Advances in the medical and surgical treatment of Takayasu arteritis. Curr Opin Rheumatol. Jan 2005;17(1):16-24. [Medline].

  24. Kumar S, Mandalam R, Rao VRK. Percutaneous transluminal angioplasty in non-specific aortoarteritis (Takayasu's disease): experience in 16 cases. Cardiovasc Intervent Radiol. 1990;12:321.

  25. Sharma BK, Jain S, Bali HK. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int J Cardiol. 2000;75 Suppl 1:S147-52. [Medline].

  26. Tyagi S, Kaul UA, Arora R. Endovascular stenting for unsuccessful angioplasty of the aorta in aortoarteritis. Cardiovasc Intervent Radiol. 1999;22:452-6. [Medline].

  27. Tyagi S, Singh B, Kaul UA. Balloon angioplasty for renovascular hypertension in Takayasu''s arteritis. Am Heart J. 1993;125:1386-93. [Medline].

  28. Bali HK, Bhargava M, Jain AK. De novo stenting of descending thoracic aorta in Takayasu arteritis: intermediate-term follow-up results. J Invasive Cardiol. 2000;12.:612-7. [Medline].

  29. Shih G, Shih WJ, Huang WS, Milan PP. Hashimoto thyroiditis and Takayasu aortitis: visualization of the thyroid gland and ring appearance of the mediastinum on F-18-FDG PET. Clin Nucl Med. May 2008;33(5):377-9. [Medline].

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Further Reading

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Keywords

Takayasu arteritis, Takayasu's arteritis, Takayasu disease, Takayasu's disease, nonspecific aortoarteritis, pulseless disease, aortic arch syndrome, granulomatous vasculitis, aortic inflammation

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Contributor Information and Disclosures

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, MD, MBA 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
Disclosure: Nothing to disclose.

Medical Editor

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
Anthony Watkinson, MD is a member of the following medical societies: Radiological Society of North America, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Douglas M Coldwell, MD, PhD,, Principal, Coldwell Associates. Interventional Radiologist, Jane Phillips Medical Center, Bartlesville, OK
Douglas M Coldwell, MD, PhD, is a member of the following medical societies: American Association for Cancer Research, American College of Radiology, American Heart Association, American Physical Society, American Roentgen Ray Society, Society of Cardiovascular and Interventional Radiology, Southwest Oncology Group, and Special Operations Medical Association
Disclosure: Sirtex, Inc. Consulting fee Speaking and teaching

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Kyung J Cho, MD, FACR, William Martel Professor of Radiology, Interventional Radiology Fellowship Director, University of Michigan Health System
Kyung J Cho, MD, FACR is a member of the following medical societies: American College of Radiology, American Heart Association, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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