Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Takayasu Arteritis Workup

  • Author: Christine Hom, MD; Chief Editor: Lawrence K Jung, MD  more...
Updated: Nov 03, 2015

Approach Considerations

Takayasu arteritis (Takayasu arteritis) has no specific markers. However, the following results can be found in laboratory studies:

  • Complete blood count (CBC) reveals a normochromic, normocytic anemia in 50% of patients with Takayasu arteritis; acute phase reactants are elevated, with leukocytosis and thrombocytosis
  • Westergren erythrocyte sedimentation rate is elevated
  • Comprehensive metabolic profile may indicate elevated transaminases and hypoalbuminemia
  • The von Willebrand factor–related antigen (factor VIII–related antigen) may be elevated
  • Antiendothelial antibodies are present
  • Antinuclear antibody results are usually negative
  • Antineutrophil cytoplasmic antibody results are usually negative
  • Rheumatoid factor is elevated in 15% of individuals with Takayasu arteritis
  • Increased levels of immunoglobulins G, M, and A are present

Patients with normal study results can still have progressive disease in the large vessels, as demonstrated on pathology specimens. The biomarker pentraxin 3, a protein closely related to C-reactive protein, may be positive in some patients with active disease with a normal C-reactive protein.[15] It is synthesized locally, in response to innate immune signaling by Toll receptors and reflects treatment of disease and relapse. The biomarker matrix metalloproteinase (MMP)–9 is also synthesized locally and is elevated in active disease, compared with normal controls and inactive disease.[16] Whereas the white blood cell count and sedimentation rate decrease with prednisolone treatment, pentraxin 3 and MMP-9 do not.

Patients with ischemia in all 4 extremities may have falsely low peripheral blood pressures. Accurate blood pressure monitoring in such patients can be obtained reliably only by central systemic measurements.



Arteriography is the criterion standard for assistance in the diagnosis of Takayasu arteritis. It is performed either with invasive angiography or, more frequently, with magnetic resonance angiography (MRA). These modalities are most helpful in identifying changes in the vessel lumen. They are less successful in delineating mural disease. Drawbacks to arteriography include morbidity from the use of contrast dye in patients with renal disease and cumulative radiation exposure over time, which can be avoided by using MRA.

Arteriography often demonstrates long, smooth, tapered narrowings or occlusions. The most frequent lesions are stenotic. Stenoses occur in 90-100% of patients with Takayasu arteritis and aneurysm formation in only 27-40%. Some authors recommend arteriography of the entire aorta. Peripheral blood pressure monitoring is frequently inaccurate in persons with Takayasu arteritis; pressure readings during angiography alone may reveal aortic root hypertension.


CT Scanning and MRI

CT scanning and magnetic resonance imaging (MRI) are useful for serial examinations and diagnosis in the early phase of Takayasu arteritis. They may reveal mural thickening of the aorta and luminal narrowing.[17, 18] (See the images below.)

MRI of thorax of 15-year-old adolescent girl with MRI of thorax of 15-year-old adolescent girl with Takayasu arteritis. Note aneurysms of descending aorta.
Coronal MRI of abdomen of 15-year-old adolescent g Coronal MRI of abdomen of 15-year-old adolescent girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys.

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. (See the images below.) Ultrasound can be used for monitoring in these areas. Delayed contrast techniques can demonstrate enhancement of the aortic wall, confirmed by a pathology specimen in a patient with normal inflammatory markers.[19, 20]

Gadolinium-enhanced cardiovascular MRI has also been used to demonstrate myocardial perfusion defects, which may be important in long-term prognosis.

Noncontrast, T2-weighted, short inversion imaging recovery (STIR) images may be used to monitor edema in the aortic wall, which may be a surrogate for inflammation; edema was found in 94% of patients with clinically active disease.

Large vessel edema was found in 56% of patients in clinical remission, similar to the 42-44% of patients in clinical remission who were found to have active vasculitis on pathology from bypass specimens. The prognostic significance of vessel edema is uncertain, as progression of lesions occurs in areas without edema, and progression may be absent from areas with edema on subsequent studies.


Additional Imaging Studies

Additional imaging studies include the following[21, 22] :

  • 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG PET) scanning – This technique shows FDG uptake in active lesions, as well as other areas without mural thickening. Enhancement disappears with treatment and is independent of inflammatory mediators. The ability to identify intramural lesions at the inflammatory stage, prior to morphological changes, is becoming possible.
  • Gallium-67 radionuclide scan - This scan may demonstrate increased uptake in the aorta and branches and is useful in comparison with MRI for large lesions
  • High-resolution ultrasonography - Duplex Doppler may be used to evaluate and monitor disease in the common carotids and subclavian arteries, with carotid evaluation revealing a homogenous, circumferential thickening of the vessel wall that is distinguishable from atherosclerotic thickening; this imaging study is only useful in evaluating superficial vessels. It can be used serially to follow flow and lumen.
  • Chest radiography - Chest radiography may reveal widening of the ascending aorta, an irregular descending aorta, aortic calcifications, and rib notching (late findings)
  • Echocardiography - Perform echocardiography at baseline to evaluate the aortic valve; Perform follow-up echocardiography as indicated to monitor aortic insufficiency from aortic root dilatation.

Histologic Findings

Mononuclear infiltration of the adventitia with perivascular cuffing of the vasa vasorum occurs early in the disease.

Granulomatous changes may be observed in the tunica media with Langerhans cells and central necrosis of elastic fibers and smooth muscle cells. Later, fibrosis of the media and acellular thickening of the intima may compromise the vessel lumen. Grossly, wrinkling of the intima is found.

Histologic specimens seldom are available, due to the large vessels affected, with the exception of specimens obtained during autopsy and bypass surgery. The most common infiltrates after CD4 T cells are CD19/CD20 B cells.

Contributor Information and Disclosures

Christine Hom, MD Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College

Christine Hom, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association, Arthritis Foundation

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.


Thomas JA Lehman, MD, FAAP, FACR Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

  1. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006 Jul. 65(7):936-41. [Medline].

  2. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan. 65(1):1-11. [Medline].

  3. de Pablo P, Garcia-Torres R, Uribe N, et al. Kidney involvement in Takayasu arteritis. Clin Exp Rheumatol. 2007 Jan-Feb. 25(1 Suppl 44):S10-4. [Medline].

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

  5. Hoyer BF, Mumtaz IM, Loddenkemper K, et al. Takayasu arteritis is characterised by disturbances of B cell homeostasis and responds to B cell depletion therapy with rituximab. Ann Rheum Dis. 2012 Jan. 71(1):75-9. [Medline].

  6. Gedalia A, Cuchacovich R. Systemic vasculitis in childhood. Curr Rheumatol Rep. 2009 Dec. 11(6):402-9. [Medline].

  7. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar. 56(3):1000-9. [Medline].

  8. Ernst D, Greer M, Stoll M, Meyer-Olson D, Schmidt RE, Witte T. Remission achieved in refractory advanced takayasu arteritis using rituximab. Case Rep Rheumatol. 2012. 2012:406963. [Medline].

  9. Salvarani C, Magnani L, Catanoso MG, et al. Rescue treatment with tocilizumab for Takayasu arteritis resistant to TNF-a blockers. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S90-3. [Medline].

  10. Unizony S, Arias-Urdaneta L, Miloslavsky E, Arvikar S, Khosroshahi A, Keroack B. Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res (Hoboken). 2012 Nov. 64(11):1720-9. [Medline].

  11. Miller JH, Gunarta H, Stanley P. Gallium scintigraphic demonstration of arteritis in Takayasu disease. Clin Nucl Med. 1996 Nov. 21(11):882-3. [Medline].

  12. [Guideline] Pickering TG, Hall JE, Appel LJ, et al. Recommendations for blood pressure measurement in humans and experimental animals: part 1: blood pressure measurement in humans: a statement for professionals from the Subcommittee of Professional and Public Education of the American Heart Association Council on High Blood Pressure Research. Circulation. 2005 Feb 8. 111(5):697-716. [Medline].

  13. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010 May. 69(5):798-806. [Medline].

  14. Szugye HS, Zeft AS, Spalding SJ. Takayasu Arteritis in the pediatric population: a contemporary United States-based single center cohort. Pediatr Rheumatol Online J. 2014. 12:21. [Medline].

  15. Dagna L, Salvo F, Tiraboschi M, Bozzolo EP, Franchini S, Doglioni C, et al. Pentraxin-3 as a marker of disease activity in Takayasu arteritis. Ann Intern Med. 2011 Oct 4. 155(7):425-33. [Medline].

  16. Ishihara T, Haraguchi G, Tezuka D, Kamiishi T, Inagaki H, Isobe M. Diagnosis and assessment of Takayasu arteritis by multiple biomarkers. Circ J. 2013. 77(2):477-83. [Medline].

  17. 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. 2007 May. 45(5):906-14. [Medline].

  18. Kissin EY, Merkel PA. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol. 2004 Jan. 16(1):31-7. [Medline].

  19. Ginde S, Cava JR, Southern JF, Saudek DE. Delayed contrast-enhanced magnetic resonance imaging in the evaluation of Takayasu arteritis. J Am Coll Cardiol. 2012 Mar 20. 59(12):e23. [Medline].

  20. Jiang L, Li D, Yan F, Dai X, Li Y, Ma L. Evaluation of Takayasu arteritis activity by delayed contrast-enhanced magnetic resonance imaging. Int J Cardiol. 2012 Mar 8. 155(2):262-7. [Medline].

  21. de Leeuw K, Bijl M, Jager PL. Additional value of positron emission tomography in diagnosis and follow-up of patients with large vessel vasculitides. Clin Exp Rheumatol. 2004. 22(6 Suppl 36):S21-6. [Medline].

  22. Schmidt WA, Blockmans D. Use of ultrasonography and positron emission tomography in the diagnosis and assessment of large-vessel vasculitis. Curr Opin Rheumatol. 2005 Jan. 17(1):9-15. [Medline].

  23. Kim YW, Kim DI, Park YJ, Yang SS, Lee GY, Kim DK. Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vasc Surg. 2012 Mar. 55(3):693-700. [Medline].

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

  25. Daina E, Schieppati A, Remuzzi G. Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med. 1999 Mar 2. 130(5):422-6. [Medline].

  26. Shinjo SK, Pereira RM, Tizziani VA, Radu AS, Levy-Neto M. Mycophenolate mofetil reduces disease activity and steroid dosage in Takayasu arteritis. Clin Rheumatol. 2007 Nov. 26(11):1871-5. [Medline].

  27. Goel R, Danda D, Mathew J, Edwin N. Mycophenolate mofetil in Takayasu's arteritis. Clin Rheumatol. 2010 Mar. 29(3):329-32. [Medline].

  28. Haberhauer G, Kittl EM, Dunky A, Feyertag J, Bauer K. Beneficial effects of leflunomide in glucocorticoid- and methotrexate-resistant Takayasu's arteritis. Clin Exp Rheumatol. 2001 Jul-Aug. 19(4):477-8. [Medline].

  29. Hoffman GS, Merkel PA, Brasington RD, Lenschow DJ, Liang P. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004 Jul. 50(7):2296-304. [Medline].

  30. Karageorgaki ZT, Mavragani CP, Papathanasiou MA, Skopouli FN. Infliximab in Takayasu arteritis: a safe alternative?. Clin Rheumatol. 2007 Jun. 26(6):984-7. [Medline].

  31. Tanaka F, Kawakami A, Iwanaga N, et al. Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate. Intern Med. 2006. 45(5):313-6. [Medline].

  32. Stern S, Clemente G, Reiff A, Ramos MP, Marzan KA, Terreri MT. Treatment of Pediatric Takayasu arteritis with infliximab and cyclophosphamide: experience from an American-Brazilian cohort study. J Clin Rheumatol. 2014 Jun. 20 (4):183-8. [Medline].

  33. Schmidt J, Kermani TA, Bacani AK, Crowson CS, Matteson EL, Warrington KJ. Tumor necrosis factor inhibitors in patients with Takayasu arteritis: experience from a referral center with long-term followup. Arthritis Care Res (Hoboken). 2012 Jul. 64(7):1079-83. [Medline].

  34. Seitz M, Reichenbach S, Bonel HM, Adler S, Wermelinger F, Villiger PM. Rapid induction of remission in large vessel vasculitis by IL-6 blockade. A case series. Swiss Med Wkly. 2011 Jan 17. 141:w13156. [Medline].

  35. Bravo Mancheno B, Perin F, Guez Vazquez Del Rey Mdel M, Garcia Sanchez A, Alcazar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis. Pediatrics. 2012 Dec. 130(6):e1720-4. [Medline].

Aortogram of a 15-year-old adolescent girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery.
MRI of thorax of 15-year-old adolescent girl with Takayasu arteritis. Note aneurysms of descending aorta.
Coronal MRI of abdomen of 15-year-old adolescent girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.