Idiopathic Arteritis of Takayasu (“Pulseless Disease”)
Takayasu’s disease is a rare polyarteritis of unknown cause with a special predilection for the branches of the aortic arch. It results in segmental stenoses, occlusions, and aneurysms. It is seen frequently in Asian women under the age of 40 years. There are a myriad of clinical presentations depending on the stage of disease (early “inflammatory” or late “occlusive”) and the arteries involved. Early stages of disease are often accompanied by fever, myalgias, arthralgias, and pain over the involved artery as well as leukocytosis and elevated sedimentation rate. Other symptoms are related to arterial insufficiency: syncope, dizziness, amaurosis fugax, stroke, angina, pulmonary hypertension, and claudication. Hypertension related to proximal renal artery stenosis or aortic coarctation is present in over one-quarter of patients. Vascular bruits, diminished peripheral pulses, or asymmetric blood pressure measurements are common findings on physical examination.
Angiography is essential for diagnosis and most often will reveal combined occlusive and aneurysmal disease. The most commonly involved vessels are the subclavian artery, descending thoracic aorta, renal artery, carotid artery, and mesenteric arteries, although the ascending and abdominal aorta and the vertebral, coronary, pulmonary, iliac, and brachial arteries can also be affected.
In cases of upper extremity stenoses, aortic root pressure should be measured by manometry at the time of angiography to assess for essential hypertension. MRA can also be used for routine surveillance of patients with suspected Takayasu’s disease.
Pulseless disease must be differentiated from vascular lesions of the aortic arch due to atherosclerosis, though in the latter instance concomitant lower extremity disease is invariably present. Histologically, the arterial lesions are indistinguishable from those of giant cell arteritis. In the early, active stage, corticosteroids (prednisone, 1 mg/kg/d) are used to control symptoms and limit the progression of disease; cytotoxic agents may be added if steroids are ineffectual. Surgical or percutaneous intervention is not advised until the disease becomes indolent. Bypass of the occluded or aneurysmal segments is the usual surgical approach. Histologic examination of the resected artery may show nonspecific transmural inflammation or chronic fibrosis. Percutaneous angioplasty or stenting is possible for short-segment stenoses, but there is a high recurrence rate in these usually noncompliant vessels.
Johnston SL et al: Takayasu arteritis: a review. J Clin Pathol 2002;55:481.
Revision date: July 8, 2011
Last revised: by David A. Scott, M.D.