AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

In 1908, Mikito Takayasu, a Japanese ophthalmologist, reported the association of retinal arteriovenous anastomoses and absent upper extremity pulses. Takayasu arteritis (TA) is a chronic inflammatory disease of the aorta and its major branches. TA is a large vessel vasculitis of unknown origin that most often affects young women in the second and third decades of life. TA has been reported in children as young as 6 months and in adults of every age.

The initial complaints may be nonspecific constitutional signs and symptoms (eg, fever, weight loss, lethargy). Because these complaints lack specificity, the correct diagnosis may be delayed for months or years. Upon histologic examination, the aorta demonstrates evidence of inflammation. Mixed areas of stenosis or aneurysm formation are found on angiography or magnetic resonance angiography (MRA). Vascular insufficiency related to stenosis and thrombosis of affected vessels may cause renovascular hypertension, neurologic symptoms, or lower extremity claudications.

Cardiac involvement may include aortic regurgitation and congestive heart failure resulting from myocarditis or increased afterload. Often the diagnosis of TA is made when a widened mediastinum is appreciated on chest roentgenography and a tumor is suspected. CT scanning instead reveals a widened aortic arch.

Despite the term pulseless disease, which is a synonym for TA, the predominant finding in individuals with TA is asymmetric pulse. Absent peripheral pulses occur late in the course of the disease. Although 5-year survival rates exceed 90%, the disease has a high incidence of residual morbidity.

Pathophysiology

TA is characterized by granulomatous inflammation of the aorta and its major branches, leading to stenosis, thrombosis, and aneurysm formation. The lesions of TA are segmental with a patchy distribution.

Mononuclear infiltration of the adventitia occurs early in the course of the disease, with cuffing of the vasa vasorum. Granulomatous changes may be observed in the tunica media with Langerhans cells and central necrosis of elastic fibers and smooth muscle cells. A panarteritis with infiltrates of lymphocytes, plasma cells, histiocytes, and giant cells is present. Later, fibrosis of the media and acellular thickening of the intima compromise the vessel lumen. Wrinkling of the intima is visible upon gross examination.

Stenoses are found in 90% of patients with TA disease. Patients often have poststenotic dilatations and other aneurysmal areas. Stenotic arterial segments result in varied ischemic symptoms. These symptoms may range from abdominal pain after eating secondary to narrowing of the mesenteric arteries to renovascular hypertension to claudication of extremities. Endothelial activation leads to a hypercoagulable state predisposing the patient to thrombosis. Congestive heart failure in individuals with TA may occur as a result of hypertension, aortic root dilation, or myocarditis. Transient ischemic attacks, cerebrovascular accidents, mesenteric ischemia, carotidynia, and claudication may occur. Symptoms of vascular compromise may be minimized by the development of collateral circulation with the slow onset of stenosis. Vessel wall dissection or aneurysm may occur in areas weakened by inflammation.

One hypothesis for granulomatous vasculitis development is that antigens deposited in vascular walls activate CD4+ T cells, followed by release of cytokines chemotactic for monocytes. These monocytes are transformed into macrophages that mediate endothelial damage and granuloma formation in the vessel wall. A mouse model supports this hypothesis. When syngeneic T cells sensitized to vascular smooth muscle cells were injected into mice, a granulomatous vasculitis of the pulmonary arterioles occurred in 20% of the mice. Human studies suggesting endothelial cell activation have demonstrated increased expression of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) in patients with TA. Humoral immunity is also believed to be involved in this disease; antiaorta antibodies and antiendothelial cell antibodies have been found in patients with TA. Immunoglobulin G, immunoglobulin M, and properdin are found in lesions from pathologic specimens.

Frequency

United States

In Minnesota's Olmstead County, incidence of TA was estimated at 2.6 per million. However, the applicability of this number to the diverse population of the United States as a whole is uncertain.

International

TA is common in underdeveloped countries, where the disease is closely associated with tuberculosis. The nature of this association is unclear because most patients with TA in the United States do not have tuberculosis. In contrast, many physicians in underdeveloped countries assume tuberculosis is present in every patient with TA.

Mortality/Morbidity

Because the disease is rare in the United States, accurate survival data are uncertain. One study reported a survival rate of 85-95% at 15 years. In a 1994 study, only 2% of deaths were attributed directly to TA. Japanese studies also support 90-95% survival rates.

In contrast, in a 1991 series involving 26 Mexican children aged 3-15 years, the 5-year survival rate was only 35%.¹ Deaths resulted from rupture of aorta or aneurysms (2), stroke (2), cardiac failure (2), and peritonitis and ventricular fibrillation.

Morbidities in persons with TA are related to ischemia and hypertension and include congestive heart failure, transient ischemic attacks, stroke, and visual disturbances. Chronic low-grade dissection of the aorta may cause recurrent chest pain for years. Upon autopsy, children with TA who have died from acute rupture of the aorta are often found to have evidence of multiple prior small dissections that did not progress.

Race

TA is more common in Asian populations but has been described in patients of all races. Japanese patients with TA have a higher incidence of aortic arch involvement. In contrast, series from India report higher incidences of thoracic and abdominal involvement. In US patients with TA, the most commonly involved vessels are the left subclavian, superior mesenteric, and abdominal aorta. In US children with TA, lesions of the thoracic and abdominal aorta, rather than lesions of the aortic arch, are most common. However, all patterns of vascular involvement have been observed in every country.

Sex

In adults, females comprise 80-90% of patients with TA. Pediatric studies are more varied. Sex distribution usually mirrors the 80-90% female preponderance observed in adults. Series of studies of TA in childhood from India and South Africa report a 2:1 female-to-male ratio. However, these are countries in which TA is associated strongly with tuberculosis, and additional etiologic and pathophysiologic factors may be present.

Age

TA is the most common large vessel vasculitis of adolescence. TA is an uncommon vasculitis in children. The most common are postinfectious vasculitides, Henoch-Schönlein purpura, polyarteritis nodosa, and Kawasaki disease. Most cases of TA present in persons aged 10-30 years. In a series of patients with TA, 20-35% were younger than 20 years at diagnosis. The youngest patient reported was aged 6 months.

CLINICAL

Section 3 of 11  

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• Clinical
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History

• Systemic symptoms in Takayasu arteritis (TA) 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

• Blood pressure difference greater than 30 mm Hg between arms
• Asymmetric pulses
• Diminished or absent pulses (midaortic lesions found in children may not affect pulses)
• Asymmetric pulses (common) and absent pulses (rare), even in the later stages of the disease (awareness of this is critical)
• 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 aorta
• Funduscopic examination
• • Retinal hemorrhages
  • Cotton-wool exudates
  • Venous dilatation and beading
  • Microaneurysms of peripheral retina
  • Optic atrophy
  • Vitreous hemorrhage
  • Classic wreathlike peripapillary arteriovenous anastomoses (extremely rare)
• Reported skin lesions including erythema nodosum–like lesions, pyoderma gangrenosum, leukocytoclastic vasculitis, and panniculitis

Causes

• TA has been reported in identical twins, leading to hypotheses of a hereditary basis for disease. In Japan and Korea, TA is associated with human leukocyte antigens (HLAs)-A10, B5, Bw52, DR2, and DR4. These associations have not been confirmed in Western studies. TA is associated with HLA-B22 in the United States.
• Although early reports associated tuberculosis with TA, these involved populations endemic for tuberculosis, and population studies have not borne out this association. Others report increased incidence of positive purified protein derivative relative to control patients. The true nature of the association with tuberculosis (if any) is unclear. Patients with TA do not improve with antituberculous treatment.

DIFFERENTIALS

Section 4 of 11  

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• Clinical
• Differentials
• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Systemic-onset juvenile rheumatoid arthritis
Migraine
Infection
Malignancy
Cogan syndrome

WORKUP

Section 5 of 11  

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• Clinical
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• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab 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.

TREATMENT

Section 6 of 11  

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Medical Care

Medical evaluation and treatment in patients with Takayasu arteritis (TA) can be performed on an outpatient basis unless the patient is acutely ill. Goals of medical therapy are to control active inflammation and to normalize clinical and laboratory parameters while preventing further vascular damage.

• Daily high-dose corticosteroid administration is the mainstay of initial therapy. The authors have used prednisone at 1-2 mg/kg/d for 4-6 weeks.
• • Maintain high-dose treatment until all evidence of active disease has resolved.
  • Then taper prednisone dosage over a month to decrease morbidity from corticosteroid treatment; although 60% of patients respond to this treatment, 40% relapse on steroid taper.
• Patients not responding to corticosteroids or who relapse during corticosteroid taper require an additional agent.
• • Symptoms of patients who relapse on corticosteroid taper may be controlled with weekly infusions of methylprednisolone (30 mg/kg, not to exceed 1 g/wk).
  • However, extensive use of these infusions is associated with significant steroid-induced toxicity if continued for any significant period.
• Regimens including weekly methotrexate or daily or monthly intravenous cyclophosphamide have been used in individuals with glucocorticoid-resistant TA. Low-dose weekly methotrexate also has been used as a steroid-sparing agent for patients not tolerating corticosteroid taper. Ozen et al used daily oral cyclophosphamide, which was well tolerated in a small series of children.²
• Cyclosporine may be an alternative therapy offering lower ovarian toxicity than cyclophosphamide. However, cyclosporine is often associated with decreased renal function and increased blood pressure, which may aggravate the damage to the heart and great vessels and is less frequently used.
• Mycophenolate mofetil may be useful to treat individuals with glucocorticoid-resistant disease.³ Case reports suggest disease control and steroid sparing.
• A small series (15 patients) showed that tumor necrosis factor (TNF) inhibition using etanercept or infliximab was successful in inducing clinical remission and permitting corticosteroid taper in patients who are steroid dependent.⁴ The role of TNF inhibition in treating initial disease or relapses is yet to be established, but use of these agents and immunosuppressants, such as mycophenolate, anticipate regimens in which disease is controlled while minimizing morbidity from steroid and cytotoxic treatments.
• Anecdotal reports of matrix metalloproteinase inhibition using minocycline propose that this may be a useful adjunctive therapy, which may also allow lower doses of corticosteroids and thus reduced toxicity.

Surgical Care

Following the acute phase, patients with fibrotic changes require surgical treatment of symptomatic stenotic or occlusive disease. This can be achieved by percutaneous angioplasty or stenting or, in severe cases, by resection and placement of a manmade graft. Children with TA rarely require bypass surgery of carotid stenting.

Percutaneous balloon angioplasty of the aorta is reported to normalize systolic and diastolic blood pressures within 24 hours, with improvement of exercise tolerance and restoration of peripheral pulses. A high incidence of restenosis (£78%) is observed in adults. Both renovascular hypertension and congestive failure due to increased afterload are improved. Improvement has been sustained for as long as 3-5 years.

Endovascular stenting is used in patients with severe stenoses, hypertension, or ischemia during the fibrotic phase of the disease. Multiple stents have been used in children to relieve long-segment renal artery stenosis and attendant renovascular hypertension. Children with TA who have received stents have lowered arterial blood pressures and decreased requirement for antihypertensives. Immunosuppressant-eluting stents could potentially deliver local treatment at sites of inflammation.

Consultations

• Pediatric rheumatologist
• Ophthalmologist
• Pediatric cardiologist
• Vascular surgeon
• Interventional radiologist

Activity

Patient activity is generally self-limiting, based on cardiac status.

MEDICATION

Section 7 of 11  

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The mainstay of initial therapy is daily high-dose corticosteroid administration. Maintain high-dose treatment for several weeks until all evidence of active disease has resolved. Among patients receiving this treatment, 60% respond; however, 40% relapse on steroid taper.

Patients who do not respond to corticosteroids or who relapse during corticosteroid taper require a second agent. Regimens including weekly methotrexate and daily cyclophosphamide have been used in individuals with glucocorticoid-resistant Takayasu arteritis (TA). Low-dose weekly methotrexate has also been used as a steroid-sparing agent for patients not tolerating corticosteroid taper. Cyclosporine may be an alternative therapy offering lower ovarian toxicity than cyclophosphamide. Reports indicate mycophenolate mofetil may help treat glucocorticoid-resistant disease. Leflunomide has been used in glucocorticoid-resistant and methotrexate-resistant disease.⁵ Tumor necrosis factor (TNF) inhibition with etanercept or infliximab has also been used in relapsing disease or glucocorticoid-dependent disease.

Drug Category: Immunosuppressive agents

These agents are used to suppress inflammation, thus delaying progression of thrombosis, stenosis, and aneurysm.

FOLLOW-UP

Section 8 of 11  

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• Introduction
• Clinical
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• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• Monitor medications and adverse effects in patients with Takayasu arteritis (TA).
• Monitor acute phase reactants as a limited measure of disease activity.
• Perform regular imaging of affected vasculature as well as surveillance imaging for new lesions. Treatment may be monitored with MRI and/or magnetic resonance angiography (MRA) or CT scanning. Mural thickening is observed to decrease with corticosteroid treatment.
• Recognizing that TA may progress in the absence of clinical findings is important. Patients with normal erythrocyte sedimentation rates who are undergoing graft placement have been found to have active aortitis in the resected segment. Periodic imaging may identify an active disease by the appearance of new areas of stenosis, despite normal erythrocyte sedimentation rate and the absence of clinical features. The presence of active disease requires treatment with corticosteroids; current markers of disease activity are inadequate to identify all patients with disease flare.
• Serial MRI may reveal vessel wall edema, but whether this measures actual inflammation is unclear. Structural changes visible on imaging demonstrate disease progression, but reliable indicators of vessel inflammation prior to structural damage are yet to be identified.

Complications

• Congestive heart failure due to aortic insufficiency, myocarditis, and/or hypertension
• Aortic aneurysms, thrombus formation, and rupture
• Ischemic stroke
• Myocardial infarction
• Hypertension
• Clinically silent progressive disease and morbidity resulting from treatment medications (take into account with long-term treatment plans)

Prognosis

• TA is a chronic relapsing disease. More than half of patients with TA achieve control on corticosteroids alone; however, their relapse rate is high and they require long periods of steroid treatment.
• Overall prognosis in individuals with TA relates to the degree of vascular and end-organ damage, specifically retinal vasculopathy, aortic insufficiency, aortic aneurysms, and hypertension.
• Survival rate at 15 years is as high as 95%.
• Of patients with TA who are treated with glucocorticoids, 60% respond; however, as many as 40% relapse on tapering steroids. In the Cleveland Clinic series of 75 patients, 93% achieved remission; only 28% of patients were able to maintain 6 months of remission when steroids were tapered to 10 mg or less.⁶

Patient Education

• Review signs of corticosteroid excess (ie, Cushing syndrome) with patient and family.
• Refer patients for psychosocial counseling.
• Reinforce medication compliance.

MISCELLANEOUS

Section 9 of 11  

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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Failure to recognize underlying malignancy
• Failure to recognize signs and symptoms of Takayasu arteritis (TA)

Special Concerns

• 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.
• Early diagnosis can be difficult due to nonspecific early symptoms; perform a thorough evaluation to eliminate other diagnoses.

MULTIMEDIA

Section 10 of 11  

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• Follow-up
• Miscellaneous
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Media file 1:  Aortogram of a 15-year-old adolescent girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery.
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Media type:  Radiograph

Media file 2:  MRI of thorax of 15-year-old adolescent girl with Takayasu arteritis. Note aneurysms of descending aorta.
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Media type:  MRI

Media file 3:  Coronal MRI of abdomen of 15-year-old adolescent girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys.
	View Full Size Image
Media type:  MRI

REFERENCES

Section 11 of 11

• Authors and Editors
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• Clinical
• Differentials
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Article Last Updated: Oct 31, 2008