Pediatric Takayasu Arteritis Clinical Presentation

  • Author: Christine Hom, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Dec 6, 2011
 

History

Systemic symptoms in Takayasu arteritis include the following:

  • Fever, night sweats
  • Fatigue
  • Weight loss
  • Myalgia and/or arthralgia and/or arthritis
  • Skin rash (eg, erythema nodosum, pyoderma gangrenosum)
  • Headaches and/or dizziness and/or syncope
  • Congestive heart failure, palpitations, angina
  • Hypertension (may be paroxysmal)

Symptoms related to ischemia include the following:

  • Ischemic stroke and/or transient ischemic attack
  • Visual disturbances (eg, blurred vision, diplopia, amaurosis)
  • Carotidynia
  • Abdominal pain
  • Claudications (vary due to the development of collateral circulations; symptom is rare in children)
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Physical Examination

The following symptoms may be observed on physical examination:

  • Blood pressure difference greater than 30 mm Hg between arms[8]
  • Asymmetrical pulses
  • Diminished or absent pulses - Midaortic lesions found in children may not affect pulses
  • Poststenotic dilatations producing what appear to be bounding pulses (often present)
  • Hypertension (may be paroxysmal) - Because this typically results from renovascular compromise, this is a high-renin hypertension
  • Bruits - Especially over subclavian arteries or the aorta
  • Skin lesions - Reported skin lesions include erythema nodosum–like lesions, pyoderma gangrenosum, leukocytoclastic vasculitis, and panniculitis

Asymmetrical pulses (common) and absent pulses (rare) can be found even in the later stages of the disease (awareness of this is critical). Funduscopic examination may reveal the following:

  • Retinal hemorrhages
  • Cotton-wool exudates
  • Venous dilatation and beading
  • Microaneurysms of peripheral retina
  • Optic atrophy
  • Vitreous hemorrhage
  • Classic wreathlike, peripapillary arteriovenous anastomoses (extremely rare)
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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.

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, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

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.

References

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

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

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

  4. 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. Jan 2012;71(1):75-9. [Medline].

  5. Gedalia A, Cuchacovich R. Systemic vasculitis in childhood. Curr Rheumatol Rep. Dec 2009;11(6):402-9. [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. Miller JH, Gunarta H, Stanley P. Gallium scintigraphic demonstration of arteritis in Takayasu disease. Clin Nucl Med. Nov 1996;21(11):882-3. [Medline].

  8. [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. Feb 8 2005;111(5):697-716. [Medline].

  9. 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].

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

  13. Ozen S, Duzova A, Bakkaloglu A, 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].

  14. 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].

  15. 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].

  16. Buonuomo PS, Bracaglia C, Campana A, et al. Infliximab therapy in pediatric Takayasu's arteritis: report of two cases. Rheumatol Int. Oct 23 2009;[Medline].

  17. 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. Jul 2004;50(7):2296-304. [Medline].

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

  19. 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].

  20. 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. Jan 17 2011;141:w13156. [Medline].

  21. 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].

 
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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.
 
 
 
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