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)

Physical Examination

The following symptoms may be observed on physical examination:

Blood pressure difference greater than 30 mm Hg between arms^[13]
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, pyodermagangrenosum, leukocytoclasticvasculitis, 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)

An international pediatric rheumatology consortium consisting of the European League Against Rheumatism, (EULAR), Pediatric Rheumatology International Trials Organization (PRINTO), and the Pediatric Rheumatology European Society (PReS) released the following classification criteria for Takayasu arteritis and other vasculitis syndromes in children in Ankara, Turkey in 2008^[14]:

Angiographic evidence of vasculitis (angiography, CT scanning, or MRI) and 1 of the 5 criteria below
Pulse deficit or claudication
Bruits
Four-limb blood pressure discrepancy
Hypertension
Acute phase reactant levels elevated

These criteria provide a sensitivity of 100% and specificity of 99% compared with 85% sensitivity for adult Takayasu criteria.

A retrospective review emphasized that constitutional symptoms coupled with objective findings of diminished pulses, bruits, and hypertension should raise clinical suspicion for Takayasu Arteritis in pediatric patients. The study also added that pharmacologic therapy alone can be successful in controlling disease progression, however surgery was successful in minimizing symptoms when medical therapies failed.^[15]

Differential Diagnoses

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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Gedalia A, Cuchacovich R. Systemic vasculitis in childhood. Curr Rheumatol Rep. 2009 Dec. 11(6):402-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Miller JH, Gunarta H, Stanley P. Gallium scintigraphic demonstration of arteritis in Takayasu disease. Clin Nucl Med. 1996 Nov. 21(11):882-3. [QxMD MEDLINE Link].
Fan L, Zhang H, Cai J, et al. Clinical course and prognostic factors of childhood Takayasu's arteritis: over 15-year comprehensive analysis of 101 patients. Arthritis Res Ther. 2019 Jan 22. 21 (1):31. [QxMD MEDLINE Link].
[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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kissin EY, Merkel PA. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol. 2004 Jan. 16(1):31-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Goel R, Danda D, Mathew J, Edwin N. Mycophenolate mofetil in Takayasu's arteritis. Clin Rheumatol. 2010 Mar. 29(3):329-32. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Karageorgaki ZT, Mavragani CP, Papathanasiou MA, Skopouli FN. Infliximab in Takayasu arteritis: a safe alternative?. Clin Rheumatol. 2007 Jun. 26(6):984-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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Media Gallery

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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Pediatric Takayasu Arteritis Clinical Presentation

History

Physical Examination

References

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