AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

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

INTRODUCTION

Section 2 of 10  

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

Background

Takayasu arteritis (TA) is a chronic inflammatory disease of the large arteries, usually affecting the aorta and its large branches and the pulmonary arteries. It is one of the vasculitides and can manifest systemically, involving any or all of the major organ systems. The brain is a prime target.

Dr Mikito Takayasu first described TA in 1905, when he described a patient with a wreathlike coronary anastomosis of retinal vasculature surrounding the papilla. Since then, the disease has been reported in all parts of the world, although it appears to be more prevalent in Asians.

Pathophysiology

TA is an inflammatory disease of large- and medium-sized arteries, with a predilection for the aorta and its branches. Advanced lesions demonstrate a panarteritis with intimal proliferation.

Lesions produced by the inflammatory process can be stenotic, occlusive, or aneurysmal. All aneurysmal lesions may have areas of arterial narrowing. Vascular changes lead to the main complications, including hypertension, most often due to renal artery stenosis or, more rarely, stenosis of the suprarenal aorta; aortic insufficiency due to aortic valve involvement; pulmonary hypertension; and aortic or arterial aneurysm. Congestive heart failure is a common finding, much more so than dilated cardiomyopathy, myocarditis, and pericarditis, which also have been reported. In patients in whom the pulmonary artery is involved, the right artery appears to be affected more than the left, with patients developing pneumonia, interstitial pulmonary fibrosis, and alveolar damage.

Other pathophysiologic consequences include hypotensive ischemic retinopathy, vertebrobasilar ischemia, microaneurysms, carotid stenosis, hypertensive encephalopathy, and inflammatory bowel disease. Rarely, TA has also been associated with glomerulonephritis, systemic lupus, polymyositis, polymyalgia rheumatica, rheumatoid arthritis, Still disease, and ankylosing spondylitis.

Frequency

United States

• The prevalence is 2.6-6.4 persons per million population. The discrepancy is attributed to genetic factors and difficulty in diagnosis.
• Between 1971-1983 in Olmstead County, Minnesota, 3 cases were recorded, thus giving an annual incidence of 2.6 cases per million population.

International

• Worldwide incidence is estimated at 2.6 cases per million per year.
• Many cases of TA are reported from Southeast Asia, where the condition is believed to be most common. However, it has been reported in all parts of the world and in all racial groups.
• The prevalence in Sweden is similar to that in the United States (ie, 2.6-6.4 persons per million population).
• In the United Kingdom, the annual incidence is 0.15 case per million.

Mortality/Morbidity

• Because TA is rare, data on mortality and morbidity are limited. The site of the lesions and the degree of involvement determine the extent and the clinical severity. A National Institutes of Health (NIH) study of 60 patients with TA prospectively demonstrated a 3% mortality rate. This result was similar to data from Japan and China but markedly different from other reports (which reported mortality as high as 35%). Such disparity may reflect differences in access to care, definitions of disease activity, and indications for treatment.
• The same NIH study showed that 20% of patients had a monophasic illness, which was self-limiting; they did not require immunosuppressive treatment. In the remaining 80% of patients who did not have a monophasic illness and experienced one exacerbation, immunosuppressive therapy resulted in remission in 60%. Of these, one half experienced relapse after immunosuppressive therapy was stopped. The overall morbidity depends on the severity of the lesions and their consequences.

Race

Cases have been reported worldwide, but TA is most common in Southeast Asia and the Indian subcontinent.

Sex

TA is primarily a disease of young women. Men are rarely affected.

Age

TA primarily affects young women, especially those in the childbearing ages. Peak onset is in individuals in their 30s. Less than 15% of cases present in individuals older than 40 years.

CLINICAL

Section 3 of 10  

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

History

TA progresses through 3 stages. Thus, symptoms that clinicians encounter depend on how soon the patient presents; most patients present late, delaying the diagnosis. However, in actual practice, most patients do not fall readily into such groupings, and this 3-stage scheme is an oversimplification of the complex clinical presentation. Symptoms encountered can occur early or late in the course of the disease. In the NIH series including 60 patients observed over 20 years, only 33% of patients had constitutional symptoms (corresponding to stage 1); 18% of patients never progressed to the third stage.

• The first stage is an early systemic stage during which the patient may complain of constitutional symptoms (eg, fatigue, malaise, giddiness, fever). This stage is considered to be prevasculitic.
• The second stage is the vascular inflammatory stage, when stenosis, aneurysms, and vascular pain (carotidynia) tend to occur.
• • Symptoms characterizing the vascular inflammatory stage include fatigue, fevers, malaise, pain in extremities and joints, dyspnea, palpitations, headaches, rash (erythema nodosum or a lupuslike butterfly rash, which can be photosensitive), hemoptysis, ulceration, and weight loss.
  • Symptoms of vascular insufficiency include arm numbness, claudication in the legs (rare), blurry vision, double vision (which can be posture dependent), amaurosis fugax, stroke, transient ischemic attacks (TIAs), hemiplegia, seizures, and paraplegia.
  • The constitutional systemic symptoms and vascular symptoms may occur at the same time, rendering the classification into stages practically impossible.
• The third stage is the burned-out stage, when fibrosis sets in, and generally is associated with remission.
• • This stage does not occur in all patients, and even in patients who are in remission, relapses can occur.
  • Presumably, the burned-out stage manifests with minimal symptoms, but little supportive evidence is found in the literature.
• Special mention should be made regarding pregnant women.
• • The inflammatory activity is not enhanced by the pregnancy, but the perinatal period may be complicated by the associated symptoms.
  • Blood pressure may not be measurable due to pulselessness, thus making patient monitoring difficult, if not impossible. Often, calf pressures need to be obtained.
  • In pregnant women with TA, uncontrolled blood pressure may lead to subarachnoid/intracranial hemorrhage and subsequent seizures, eye changes, preeclampsia, aortic regurgitation, syncope, fetal complications, and nephrotic syndrome.

Physical

• The main finding is absent pulse(s) or a pulse discrepancy of greater than 30 mm Hg between the right and left arms.
• Other significant findings include the following:
• • Vascular bruits, most commonly in the carotid and abdominal arteries but also in the subclavian and femoral arteries
  • Focal neurologic deficits consistent with cerebral infarction or TIA
  • Hypertension, sometimes leading to hypertensive encephalopathy, usually due to renal artery stenosis
  • Amaurosis fugax
  • Retinal ischemia and microaneurysms
  • Myocardial infarction
  • Dilated cardiomyopathy
  • Eclampsia
  • Subarachnoid hemorrhage, probably secondary to hypertension
  • Leg edema due to renal failure secondary to renal artery stenosis and glomerulonephritis
• Less common associations have been seen with the following:
• • Ulcerative colitis
  • Sensorineural hearing loss
  • Pulmonary hemorrhage
• Blood pressure may not be measurable due to the pulselessness, making patient monitoring difficult or impossible. Often, calf blood pressures must be obtained.
• A case report of sensorineural hearing loss associated with TA also has been reported. Whether a firm connection between the two syndromes exists, other than the inflammatory manifestations, has not been demonstrated. The pathologic etiology of the hearing loss was attributed to an immune-mediated process in the membranous labyrinth.
• A case report of Cogan syndrome with TA also has been reported. Cogan syndrome, first described in 1945, involves interstitial keratitis and a vestibuloauditory syndrome. Variations of this with other types of inflammatory eye disease and vestibuloauditory arteritis have been reported.

Causes

The etiology is unknown. The underlying pathologic process is inflammatory, with several etiologic factors having been proposed, including spirochetes, Mycobacterium tuberculosis, streptococcal organisms, and circulating antibodies due to an autoimmune process.

An antigen may stimulate aortic tissue, leading to the expression of heat shock protein-65, which, in turn, induces major histocompatibility (MHC) class I–related chain A (MICA). Natural killer cells and gamma-delta T cells expressing NKG2D receptors may infiltrate and recognize MICA on vascular smooth muscle cells, releasing porfrin and leading to acute inflammation. Proinflammatory cytokines are also released from the natural killer and T-cells, inducing the production of matrix metalloproteinases (MMPs) and amplifying the inflammatory response. This, in turn, would induce more MHC antigen and stimulate molecule expression on vascular cells, recruiting more mononuclear cells.

• In a case report, the role of M tuberculosis and its 65-kd heat shock protein has been implicated in the etiology. Patients with TA were found to have higher immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA) titers against the M tuberculosis extract than patients without the condition.
• A recent article reports the presence of CD3⁺ T cells and IgG antibodies reactive to circulating antimycobacterial heat shock protein 65 (mHSP65) antibodies and to its human homologue, hHSP60. This suggests a possible cross reactivity of immune response between mHSP65 and hHSP60.
• Other case reports involving the role of antiendothelial cell, anticardiolipin, and antiaorta antibodies also exist.
• In Japanese patients, human leukocyte antigen Bw52 (HLA-Bw52), which is in linkage disequilibrium with human leukocyte antigen DR4 (HLA-DR4), has been observed with increased frequency. Patients with the Bw52 genotype had a higher rate of aortic regurgitation. However, studies of HLA antigens in North American populations have not confirmed this.
• A recent study demonstrated an association between several cases of TA and CD36 deficiency (CD36d). The human CD36 antigen is a multifunctional membrane glycoprotein that belongs to the class B scavenger receptor family. It is expressed on monocytes, platelets, and endothelial cells, and contributes to myocardial fatty acid transport. In patients with CD36d, myocardial I-15-(p-iodophenyl)-3-(R,S)-methyl pentadecanoic acid (BMIPP) uptake was absent.

DIFFERENTIALS

Section 4 of 10  

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

Other Problems to be Considered

Cogan syndrome
Dilated cardiomyopathy
Erythema nodosum
Relapsing polychondritis
Amaurosis fugax
Ocular pathology
Giant cell arteritis
Other arteritides
Other large vessel diseases (eg, neurofibromatosis of the abdominal aorta)
Carotid disease and stroke

WORKUP

Section 5 of 10  

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

Lab Studies

• Laboratory tests in individuals with TA tend to be nonspecific. The erythrocyte sedimentation rate (ESR) may be high, generally greater than 50 mm/h, in early disease but normal later. Leukocyte count may be normal or slightly elevated. A moderate, normochromic anemia may be present in individuals with active disease.
• Autoantibodies observed in other connective tissue diseases, including rheumatoid factor, antinuclear antibodies, anticardiolipin antibodies, and antineutrophil cytoplasmic antibodies (ANCA), are as common as in the general population. Circulating antiendothelial antibodies may be present in high titers. This finding is considered nonspecific, because it is reported sporadically and may be present in other connective tissue diseases and in angiitis obliterans. Antiaorta antibodies may be present, but testing for them seldom is performed, if ever.
• Some researchers have found that the levels of soluble vascular cell adhesion molecule-1 (VCAM-1) were significantly higher in patients with TA compared with normal healthy controls, and they were also significantly higher in older than younger patients, suggesting that VCAM-1 may serve as a marker of disease activity and progression with age.
• Hypoalbuminemia and increased levels of fibrinogen, alpha2-globulin, and gamma globulin are common.
• Urinalysis may be consistent with nephrotic syndrome.
• HLA typing has not confirmed any definite association in North American patients. Presumably, a finding of HLA-Bw52 in such patients reinforces the diagnosis; it is not a definite diagnostic tool.

Imaging Studies

• Angiography is the criterion standard.
• The Ishikawa criteria (1986) have been useful in defining TA.
• • One criterion is age younger than 40 years at diagnosis or at onset of characteristic signs and symptoms of 1-month duration in the patient's history.
  • Two major criteria involve the left and right midsubclavian artery, with the most severe stenosis or occlusion present in the mid portion from a point 1 cm proximal to the left and right, respectively, vertebral artery orifices to that 3 cm distal to the orifice as determined by angiography.
  • The minor criteria consist of annuloaortic ectasia or aortic regurgitation as shown by angiography or echocardiography and pulmonary artery, left mid common carotid, distal brachiocephalic trunk, descending aorta, or abdominal aorta lesions.
• The American College of Rheumatology (ACR; 1990) states the following:
• • Angiographic criteria must show narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities.
  • These changes are not due to arteriosclerosis, fibromuscular dysplasia, or similar causes.
  • Changes are usually focal or segmental.
  • These criteria probably allow greater flexibility to account for variability in actual clinical practice.
  • In comparison to the Ishikawa criteria, which were established based on Japanese patients only, the ACR criteria may better reflect the North American population.
  • The lesions can include stenosis, occlusion, or aneurysms.
• Computed tomography (CT) scanning or ultrasound may be used to assess thickness of the aorta.
• Magnetic resonance angiography (MRA) can be used to assess the vasculature, but it is not as accurate as angiography.
• Gallium scanning has been used to assess inflammatory involvement of the vessels.
• Single-photon emission computed tomography (SPECT) scanning has been used to assess cerebral blood flow and may be useful in patients who undergo bypass surgery. Several recent studies have shown that the use of whole-body positron emission tomography (PET) scanning provides useful information demonstrating anatomic changes consistent with the diagnosis of TA.
• Ultrasonography has been used to determine flow characteristics in stenosed vessels.
• Recent reports of the use of positron emission tomography (PET) in various vasculitic conditions, including TA, demonstrate abnormal uptake patterns of 2-[18F]fluoro-2-deoxy-D-glucose (18F-FDG) in the walls of the involved vessels. 18F-FDA accumulates in inflammatory cells due to augmented oxidative metabolism, and this may be of value in diagnosis in the early stages of the disease and for evaluating response to therapy.

Procedures

• Few procedures are necessary. Grafts have been used to bypass regions of severe stenosis or occlusion. Usually, the graft is a saphenous vein graft. Examples of grafts performed include bypass of renal artery stenosis for renal salvage; bypass of innominate or carotid artery; and bypass between subclavian-axillary and common carotid arteries. Extraintracranial bypass operations generally are performed for stenosis of the internal carotid or middle cerebral arteries.
• Other procedures include aneurysm clipping and revascularization.

Histologic Findings

• Lesions are initially inflammatory and later become occlusive. In the early phase of TA, histologic features include granulomatous changes in the media and adventitia of the aorta and its branches, followed by intimal hyperplasia, medial degeneration, and adventitial fibrosis of the sclerotic type. The duration is variable. Inflammatory cells—predominantly CD4 and CD8 lymphocytes, macrophages, plasma cells, histiocytes, and giant cells—invade the adventitia and media but not the intima.
• In the vasoocclusive stage, the lesions are characterized by occlusion and signs of ischemia. The adventitia and media are replaced by fibrous scarring, the vasa vasorum are obliterated, and the intima undergoes irregular thickening. Medial degeneration, disruption of the elastic lamellae, and thrombosis can occur. Aneurysms can form, but no aneurysms attributed to TA have been identified in the intracranial circulation. The literature reports a few cases of intracranial aneurysms that are considered to be incidental.
• The ground substance in the intima is increased markedly, histochemically showing a basophilic acid mucopolysaccharide in a state of gelatinous swelling.
• An increase in CD4 and decrease in CD8 lymphocytes, along with reduced B lymphocytes, have suggested a defect in T-cell regulation (cell-mediated immunity). Biopsy samples exhibit infiltrates of lymphocytes and monocytes in both the vessel walls and a peripheral nerve vasculitis. Lymphocytes and monocytes are attracted to the vessel wall either by an infectious agent or an autoimmune response, modulated by intercellular adhesion molecules (ICAMs).

TREATMENT

Section 6 of 10  

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

Medical Care

Medical management depends on the disease activity and the complications that can develop. Some patients have only mild forms of TA; others deteriorate considerably. The two most important aspects of treatment are controlling the inflammatory process and controlling the hypertension. Corticosteroids are the most important therapeutic agents and are necessary in active disease. Therapy is continued until patients achieve remission.

• For patients who do not achieve remission on corticosteroids, cytotoxic agents such as methotrexate (0.3 mg/kg/wk) or cyclophosphamide (1 mg/kg/d) may prove effective; azathioprine (1 mg/kg) is another possible option. For relapses, combinations of the above can be tried.
• Hypertension is treated with antihypertensive agents, and aggressive therapy is necessary to prevent complications.
• Antiplatelet agents and heparin may prove useful in preventing stroke. Warfarin also has been used. The literature reports a case of improvement in renal and systemic function with low-dose intravenous (IV) heparin therapy (10,000 U/d) followed by oral anticoagulant and antiplatelet agents.
• Anti–tumor necrosis factor (TNF) agents have recently shown encouraging results in a small number of patients with relapsing TA. Anti-TNF may be a useful adjunct to glucocorticoid therapy; however, to date, a controlled clinical trial is lacking.

Surgical Care

Bypass surgery has been performed on patients with critical thoracic aortic arch arterial stenosis, upper and lower extremity ischemia, cerebrovascular accidents, and renal artery stenosis. The procedures are generally case specific. Certain issues, such as the timing of surgery in relation to disease activity or the advisability of surgery in symptom-free patients, have not been resolved. Anastomotic stenoses or graft occlusion is a potential complication of surgery. Short lesions respond well to percutaneous transluminal angioplasty.

Consultations

• Cardiologist
• Rheumatologist
• Obstetrician-gynecologist for pregnant patients
• Vascular surgeon

Diet

Diet modification is necessary to manage hypertension or renal failure.

Activity

Any limitations depend on the severity of the disease and complications.

MEDICATION

Section 7 of 10  

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

The goals of therapy are to reduce inflammation and suppress autoimmune disease. To treat the active disease, corticosteroids are used and gradually tapered. Cytotoxic agents are the main therapeutic agents when the response to steroids is unsatisfactory.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Drug Category: Cytotoxic agents

In patients not responding to prednisone, cyclophosphamide or methotrexate—either in combination with prednisone or alone—can be used to suppress the active disease.

Drug Category: Antihypertensive agents

Can be used to treat hypertension associated with arteritis. On occasion, combinations are required. Therapy can be individualized.

Drug Category: Antiplatelet agents

Help prevent CVAs and improve renal and systemic function. A formulation of extended-release dipyridamole and aspirin (Aggrenox) also recently has become available.

Drug Category: Anticoagulants

In patients with renal failure due to crescentic glomerulonephritis and nephrotic syndrome, low-dose heparin followed by oral anticoagulation leads to improved renal and systemic function. It probably reduces the destructive effects of fibrin thrombi in the small vessels of the kidney.

Drug Category: Immunosuppressant agents

Inhibit key factors responsible for immune reactions.

FOLLOW-UP

Section 8 of 10  

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

Further Inpatient Care

• Management is long-term. Inpatient care is limited to managing acute manifestations of the disease, usually resulting in complications from organ failure, stroke, complications of pregnancy, seizures, and intracranial hemorrhage.
• ICU admission is indicated for patients with critical deterioration.
• Fetal monitoring is indicated in patients with suspected complications.

Further Outpatient Care

• Monitoring long-term immunosuppressive therapy and hypertension control is necessary.

Complications

• Complications include stroke, intracranial hemorrhage, seizures, graft stenosis and/or occlusion, ischemia, organ failure, complications of hypertension, and fetal injury.
• Long-term use of corticosteroids can lead to infection, adrenal suppression, cataracts, hyperglycemia, hypertension (which complicates blood pressure control), osteoporosis, and aseptic necrosis.

Prognosis

• Prognosis varies according to the severity of the disease and the response to therapy. In some series, mortality is high. Two main North American series showed survival figures of greater than 90%.

Patient Education

• Patients need to understand the nature of the disease and the need to take medications to prevent complications. When in remission or in mild forms, patients are tempted to stop the antihypertensive drugs, thus increasing risk of serious neurologic and other systemic complications.

MISCELLANEOUS

Section 9 of 10  

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

Medical/Legal Pitfalls

• TA is rare and difficult to diagnose. Initially, symptoms are vague; disease may progress considerably until the angiogram is performed. Some individuals may find fault with the provider if they feel diagnosis was delayed.

Special Concerns

• Pregnancy is an important concern; aggressively treat these patients.

REFERENCES

Section 10 of 10

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

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• de Jonge HJ, Knipscheer RJ, Weigel HM. Takayasu''s or pulseless disease in pregnancy. Eur J Obstet Gynecol Reprod Biol. Jan 1983;14(4):241-9. [Medline].

Article Last Updated: Dec 8, 2006