eMedicine Specialties > Rheumatology > Vasculitis

Takayasu Arteritis: Differential Diagnoses & Workup

Author: Mohammed Mubashir Ahmed, MD, Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine
Coauthor(s): Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Contributor Information and Disclosures

Updated: Feb 14, 2008

Differential Diagnoses

Other Problems to Be Considered

Fibromuscular dysplasia
Spondyloarthropathies
Thromboembolism
Traumatic stenosis
Radiation fibrosis
Ergotism
Ehlers-Danlos syndrome
Marfan syndrome
Neurofibromatosis
Syphilis
Cogan syndrome

Workup

Laboratory Studies

• The acute phase reactants and clinical parameters generally used to define active inflammatory disease do not universally reflect active blood vessel inflammation in Takayasu arteritis. The erythrocyte sedimentation rate is elevated in most but not all patients during active inflammatory disease. However, it is not a consistently reliable marker of disease activity, with both false-positive and false-negative associations being reported.
• Other markers, including endothelin-1, von Willebrand factor, factor VIII antigen, and thrombomodulin, have been found to be inadequate markers of disease activity.

Imaging Studies

• While imaging studies (CT scan, MRI) show typical patterns of stenoses or aneurysms of the arteries, angiography remains the criterion standard for diagnosis and evaluation of the extent of disease. However, recent studies suggest that noninvasive imaging modalities such as MRI, ultrasonography, and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) allow diagnosis of Takayasu arteritis earlier in the disease course than standard angiography and provide a means for monitoring disease activity.
  • Angiography can evaluate only the appearance of the lumen and cannot differentiate between active and inactive lesions.
  • Takayasu arteritis can be divided into 6 types based on angiographic involvement.
    • Type I - Branches of the aortic arch
    • Type IIa - Ascending aorta, aortic arch, and its branches
    • Type IIb - Type IIa region plus thoracic descending aorta
    • Type III - Thoracic descending aorta, abdominal aorta, renal arteries, or a combination
    • Type IV - Abdominal aorta, renal arteries, or both
    • Type V - Entire aorta and its branches
• Other tests
  • Magnetic resonance angiography (MRA) is equally or more sensitive than angiography for revealing lesions in the aorta and its brachycephalic branches but is less sensitive for helping detect smaller branch involvement. MRA that uses fast spin-echo sequences designed to enhance the detection of vessel wall edema has shown promise in assessing disease activity before irreversible lesions develop.
  • CT helical scanning angiography also has been shown to be sensitive and specific as a diagnostic tool.
  • Color Doppler ultrasonography provides details of the vascular wall, lumen, and flow and is a useful tool for screening and follow-up, particularly for carotid and subclavian arteries.
  • Positron emission tomography (PET) scanning with radioactive-labeled 18-fluorodeoxyglucose (FDG) has been shown to be useful in monitoring disease activity and response to treatment in preliminary studies. Presence or absence of FDG uptake correlates well with clinical state and MRI findings. Its use in patients with Takayasu arteritis requires further investigation.
  • MRA or CT angiography and FDG-PET may be useful to monitor vascular response to treatment.

Procedures

• Biopsy of medium- to large-sized vessels may be diagnostic in early stages of the disease; however, in the chronic phase, diagnosis by biopsy alone is inadequate. In contrast to other vasculitides, tissue biopsies play little to no role in the diagnosis of Takayasu arteritis, as histologic examination of the great vessels is usually possible only at the time of vascular procedures or postmortem. Whenever possible, the feasibility of submitting some arterial tissue should be discussed with the attending surgeon prior to any surgical revascularization procedure.
• Percutaneous balloon angioplasty can provide good outcomes for short lesions.
• Conventional stents seem to be associated with high failure rate in patients with Takayasu arteritis.
• Bypass graft surgery is the procedure with the best long-term patency rate.

Histologic Findings

Takayasu arteritis is characterized by a special pattern of histopathological changes. The early stage consists of a continuous or patchy granulomatous inflammatory reaction involving macrophages, lymphocytes, and multinucleated giant cells. Inflammation initially occurs in the vasa vasorum, with the artery wall becoming irregularly thickened and the lumen becoming narrowed. Takayasu arteritis progresses to a sclerotic stage, with intimal and adventitial fibrosis and scarring of the media.

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