eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Takayasu Arteritis: Differential Diagnoses & Workup

Author: Gabriel Bucurescu, MD, MS, Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center
Contributor Information and Disclosures

Updated: Sep 17, 2009

Differential Diagnoses

Cerebral Aneurysms
Sarcoidosis and Neuropathy
Churg-Strauss Disease
Subarachnoid Hemorrhage
Intracranial Hemorrhage
Syncope and Related Paroxysmal Spells
Lacunar Syndromes
Wegener Granulomatosis
Neurofibromatosis, Type 1
Neurofibromatosis, Type 2
Polyarteritis Nodosa

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
Reversible posterior leukoencephalopathy syndrome
Common variable immunodeficiency syndrome
Keratouveitis
Posterior scleritis
Polymyalgia rheumatica
Subclavian steal syndrome
Raynaud phenomenon

Workup

Laboratory Studies

  • Laboratory tests in individuals with Takayasu arteritis (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. One study found that multi-slice CT was useful in detecting lesions.7
  • Magnetic resonance angiography (MRA), especially whole-body studies, can be used to assess the vasculature. Recent advances in technology have substantially improved sensitivity and specificity, and the entire arterial vasculature can be displayed in less than 90 seconds. The risks are practically nonexistent, and the versatility of the images obtained will likely lead to increased use over conventional angiography.8
  • 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.
  • Angioplasty and stenting have been used to treat recurrent stenosis.

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).

More on Takayasu Arteritis

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

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Keywords

aortic arch arteritis, aortitis syndrome, pulseless disease, brachiocephalic arteritis, occlusive thromboarteritis, nonspecific arteritis, occlusive thromboaortopathy, TA

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Contributor Information and Disclosures

Author

Gabriel Bucurescu, MD, MS, Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center
Gabriel Bucurescu, MD, MS is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, and American Epilepsy Society
Disclosure: Nothing to disclose.

Medical Editor

Sydney Louis, MB, BCh, MD, Emeritus Professor, Department of Neurology, Brown University School of Medicine
Sydney Louis, MB, BCh, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

,, Kathy Roarty Placeholder
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

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