eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Takayasu Arteritis: Differential Diagnoses & Workup

Author: Christine Hom, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College
Contributor Information and Disclosures

Updated: Oct 31, 2008

Differential Diagnoses

Acute Lymphoblastic Leukemia
Behcet Syndrome
Fever Without a Focus
Hodgkin Disease
Polyarteritis Nodosa
Rheumatic Fever

Other Problems to Be Considered

Systemic-onset juvenile rheumatoid arthritis
Migraine
Infection
Malignancy
Cogan syndrome

Workup

Laboratory Studies

  • Takayasu arteritis (TA) has no specific markers.
  • CBC count reveals a normochromic normocytic anemia in 50% of patients with TA. 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 is usually negative.
  • Rheumatoid factor is elevated in 15% of individuals with TA.
  • Increased levels of immunoglobulins G, M, and A are present.

Imaging Studies

  • Arteriography either by invasive angiography or by magnetic resonance angiography (MRA)
    • Arteriography is the criterion standard for assisting in making the diagnosis of TA. However, the use of MRA is rapidly increasing.
    • Peripheral blood pressure monitoring is frequently inaccurate in persons with TA; pressure readings during angiography alone may reveal aortic root hypertension.
    • Drawbacks to arteriography, including morbidity from use of contrast dye in patients with renal disease and cumulative radiation exposure over time, can be avoided by using MRA.
    • Arteriography often demonstrates long, smooth, tapered narrowings or occlusions. Stenoses occur in 90-100% of patients with TA and aneurysm formation in only 27%. Three-dimensional MRA imaging of the aorta and its branches are providing exciting new data that may improve the understanding of the disease.
    • Some authors recommend arteriography of the entire aorta.
  • MRI, MRA, CT scanning
    • These examinations are useful for serial examinations and diagnosis in the early phase of TA.
    • CT scanning and MRI may reveal 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.
    • 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.
    • Edema was found in 56% of patients in clinical remission, similar to the 42-44% of patients found to have active vasculitis on pathology from bypass specimens from patients who were in clinical remission. 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.
  • Gallium-67 radionuclide scan: This scan may demonstrate increased uptake in the aorta and branches.
  • High-resolution ultrasonography
    • Duplex Doppler 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.
    • Carotid evaluation reveals a homogenous circumferential thickening of the vessel wall that is distinguishable from atherosclerotic thickening.
  • Chest radiography: Chest radiography may reveal widening of the ascending aorta, irregular descending aorta, aortic calcifications, and rib notching (late findings).

Other Tests

  • Perform echocardiography at baseline to evaluate the aortic valve.
  • Perform follow-up echocardiography as indicated to monitor aortic insufficiency.

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 exceptions of specimens obtained during autopsy and bypass surgery.

More on Takayasu Arteritis

Overview: Takayasu Arteritis
Differential Diagnoses & Workup: Takayasu Arteritis
Treatment & Medication: Takayasu Arteritis
Follow-up: Takayasu Arteritis
Multimedia: Takayasu Arteritis
References
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References

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

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

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

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

  5. 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. Jul-Aug 2001;19(4):477-8. [Medline].

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

  7. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. Aug 1990;33(8):1129-34. [Medline].

  8. Arora P, Kher V, Singhal MK, et al. Renal artery stenosis in aortoarteritis: spectrum of disease in children and adults. Kidney Blood Press Res. 1997;20(5):285-9. [Medline].

  9. Baumgartner D, Sailer-Hock M, Baumgartner C. Reduced aortic elastic properties in a child with Takayasu arteritis: case report and literature review. Eur J Pediatr. Nov 2005;164(11):685-90. [Medline].

  10. 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].

  11. 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].

  12. de Pablo P, Garcia-Torres R, Uribe N, Ramon G, Nava A, Silveira LH. Kidney involvement in Takayasu arteritis. Clin Exp Rheumatol. Jan-Feb 2007;25(1 Suppl 44):S10-4. [Medline].

  13. Della Rossa A, Tavoni A, Merlini G, Baldini C, Sebastiani M, Lombardi M. Two Takayasu arteritis patients successfully treated with infliximab: a potential disease-modifying agent?. Rheumatology (Oxford). Aug 2005;44(8):1074-5. [Medline].

  14. Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum. Apr 1994;37(4):578-82. [Medline].

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

  16. Karam EZ, Muci-Mendoza R, Hedges TR 3rd. Retinal findings in Takayasu's arteritis. Acta Ophthalmol Scand. Apr 1999;77(2):209-13. [Medline].

  17. Kern P, Kalden JR, Manger B. Inflammatory arterial stenosis: followup with color Doppler sonography. Arthritis Rheum. Jan 2000;43(1):238. [Medline].

  18. Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. Nov 1995;21(4):1041-58. [Medline].

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

  20. Lambert M, Hatron PY, Hachulla E, Warembourg H, Devulder B. Takayasu's arteritis diagnosed at the early systemic phase: diagnosis with noninvasive investigation despite normal findings on angiography. J Rheumatol. Feb 1998;25(2):376-7. [Medline].

  21. Lamprecht P, Till A, Steinmann J, Aries PM, Gross WL. Current State of Biologicals in the Management of Systemic Vasculitis. Ann N Y Acad Sci. Sep 2007;1110:261-270. [Medline].

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

  23. Limsuwan A, Khowsathit P, Pienvichit P. Left main coronary occlusion from Takayasu arteritis in an 8-year-old child. Pediatr Cardiol. May-Jun 2007;28(3):234-7. [Medline].

  24. Martini A. Behcet's disease and Takayasu's disease in children. Curr Opin Rheumatol. Sep 1995;7(5):449-54. [Medline].

  25. Morales E, Pineda C, Martinez-Lavin M. Takayasu's arteritis in children. J Rheumatol. Jul 1991;18(7):1081-4. [Medline].

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

  27. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. Jul 2006;65(7):936-41. [Medline].

  28. Perniciaro CV, Winkelmann RK, Hunder GG. Cutaneous manifestations of Takayasu's arteritis. A clinicopathologic correlation. J Am Acad Dermatol. Dec 1987;17(6):998-1005. [Medline].

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

  30. Shetty AK, Stopa AR, Gedalia A. Low-dose methotrexate as a steroid-sparing agent in a child with Takayasu's arteritis. Clin Exp Rheumatol. May-Jun 1998;16(3):335-6. [Medline].

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

  32. Sun Y, Yip PK, Jeng JS, Hwang BS, Lin WH. Ultrasonographic study and long-term follow-up of Takayasu's arteritis. Stroke. Dec 1996;27(12):2178-82. [Medline].

  33. Tanaka F, Kawakami A, Iwanaga N, Tamai M, Izumi Y, Aratake K. Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate. Intern Med. 2006;45(5):313-6. [Medline].

  34. Tyagi S, Kaul UA, Nair M, Sethi KK, Arora R, Khalilullah M. Balloon angioplasty of the aorta in Takayasu's arteritis: initial and long-term results. Am Heart J. Oct 1992;124(4):876-82. [Medline].

  35. Tyagi S, Sharma VP, Arora R. Stenting of the aorta for recurrent, long stenosis due to Takayasu's arteritis in a child. Pediatr Cardiol. May-Jun 1999;20(3):215-7. [Medline].

  36. Uthman IW, Bizri AR, Hajj Ali RA, Nasr FW, Khalil IM. Takayasu's arteritis presenting as fever of unknown origin: report of two cases and literature review. Semin Arthritis Rheum. Feb 1999;28(4):280-5. [Medline].

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

  38. Yamaoka K, Saito K, Nakayamada S, Yamamoto M, Tanaka Y. Clinical images: Takayasu arteritis diagnosed by positron emission tomography. Arthritis Rheum. Jul 2007;56(7):2466. [Medline].

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Further Reading

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Keywords

Takayasu arteritis, TA, Takayasu's arteritis, Takayasu disease, Takayasu's disease, Takayasu syndrome, Takayasu's syndrome, pulseless disease, nonspecific aortoarteritis, reverse coarctation, aortic arch syndrome, aortitis syndrome, vascular insufficiency, myocarditis, aortic regurgitation, thrombosis, hypertension, aortic root dilation, mesenteric ischemia, carotidynia, granulomatous vasculitis, tuberculosis, stroke, cardiac failure, ventricular fibrillation, erythema nodosum–like lesions, pyoderma gangrenosum, leukocytoclastic vasculitis, panniculitis, syncope

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Contributor Information and Disclosures

Author

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, and Arthritis Foundation
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting; Pfizer Honoraria Consulting

Chief Editor

Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association
Disclosure: Nothing to disclose.

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