History
In 1905, Mikito Takayasu, an ophthalmologist, described a 21-year-old Japanese woman with a peculiar retinal arteriovenous anastomosis, and Onishi described a patient with similar funduscopic findings and absence of radial pulses. Giovan Morgagni, an Italian pathologist, reported the first case with signs and symptoms consistent with Takayasu arteritis. In 1948, Shimizu and Sano described a condition characterized by absent pulses, peripapillary arteriovenous anastomosis of the retina, and accelerated carotid sinus reflex, which they called "pulseless disease." The name "Takayasu's disease" was applied by Caccamis in 1954.
In a study of 104 Italian patients (91 female, 13 male) with Takayasu arteritis there was a median delay in diagnosis of 15.5 months. [9] The main clinical features and laboratory findings were arterial bruit (90%), decreased or absent pulse (85%), limb blood pressure difference over 10 mm Hg (70%), claudication of extremities (45%), hypertension (40%), asthenia (50%), fever (30%), arthralgia/arthritis (25%), weight loss over 5 kg (20%), headache (20%), erythrocyte sedimentation rate greater than 30 mm/hr (85%), anemia (60%), and leukocytosis (20%). Vascular involvement based on full aortography revealed involvement of the left subclavian (65%), right subclavian (52%), left carotid (44%), abdominal aorta (39%), and right carotid (36%) arteries. [9]
Many patients have ischemia of the upper extremities that may manifest as arm claudication or numbness at the time of disease recognition. Claudication of the lower limbs is less common as a presenting symptom.
Hall et al reported arthralgias or myalgias in about one half of patients at the early stage of disease. [10] Symmetric inflammatory polyarthritides resembling rheumatoid arthritis were observed in 5 of 32 patients. Articular symptoms were either transient or continual for several months or longer. Myalgia sometimes dominates the clinical presentation and may mislead clinicians.
Neurologic symptoms are generally caused by decreased cerebral blood flow in the carotid and vertebral arteries. Neurologic manifestations include vertigo, syncope, orthostasis, headache, convulsion, transient ischemic attack, stroke, and dementia. Seizures are often attributed to hypertensive encephalopathy. Because of central retinal hypoperfusion, visual impairment is most often bilateral, and 48% of patients with vertebral artery involvement and 40% with common carotid artery involvement have visual aberrations.
In a minority of cases (8%-18% of pooled series), skin lesions resembling erythema nodosum or pyoderma gangrenosum were found on the lower extremities. Upon biopsy, the lesions frequently showed vasculitis of the small vessels. Erythema nodosum is the predominant dermatologic finding in the United States and Europe, whereas pyoderma gangrenosum is found more frequently in Japan. Raynaud phenomenon has also been reported in 8%-14% of patients.
Patients with noninfectious aortitis may present with atypical symptoms that result in diagnostic delay. Spanish researchers suggested "red flags" for the presence of aortitis may include atypical features of polymyalgia rheumatica, unexplained low back or limb pain, and constitutional symptoms in association with elevated levels of acute phase reactants. [11]
Angina pectoris may occur as a result of coronary artery ostial narrowing from aortitis or coronary arteritis and can lead to myocardial infarction, heart failure, or sudden death. Congestive heart failure may be caused by valvular disease. Aortic regurgitation that results from dilation of the aortic root is common.
In cases of documented pulmonary artery involvement, fewer than 25% of patients had related clinical manifestations and only 20% had pulmonary hypertension. Pulmonary symptoms include cough, dyspnea, and hemoptysis.
Abdominal pain, diarrhea, and gastrointestinal hemorrhage may result from mesenteric artery ischemia, but this is rare.
Specific arteries that are inflamed may be tender to the touch (eg, carotid, temporal).
Physical Examination
Patients frequently appear chronically ill. Mild to moderate fever may be present. Heart rate and rhythm are unaffected. Reduced blood pressure in one or both arms is common. Laterality of blood pressure (ie, a difference between left and right arms greater than 10 mm Hg) suggests vascular obstruction. Maneuvers can distinguish aortitis-induced pressure drop and/or pulse weakness from scalenus anticus syndrome, in which arm elevation and turning of the head precipitate symptoms.
Arterial pulse intensity in any of the limbs may be diminished, often asymmetrically. Bruits may be audible over the carotid arteries, abdominal aorta, and sometimes the subclavian and brachial arteries. In a North American study by Kerr et al, bruit was the most common clinical finding (80%), and the most common site was in the carotid vessels (70%). [12] A diastolic decrescendo murmur may signal aortic valve insufficiency. Rales, edema, liver congestion, elevated venous pressure, and hepatojugular reflux, if present, signify the complication of heart failure.
Hypertension develops in 33%-76% of patients, most frequently resulting from narrowing of the renal artery, but narrowing and decreased elasticity of the aorta and branches also can be exacerbating factors. As narrowing or occlusion may diminish the blood pressure in the arms, the pressure in all limbs must be checked, and occasionally measuring central arterial pressure by catheterization may be required to assess hypertension.
Synovitis mimicking rheumatoid arthritis may be noticeable over larger joints, such as the knees or wrists, early in the course of disease.
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Aortitis. This chart represents the presence of an associated morbidity in Takayasu arteritis in the United States (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
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Aortitis. The frequency of vascular involvement in Takayasu arteritis is depicted (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
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Aortitis. Diffuse stenosis from the aortic arch to the abdominal aorta. The left common carotid artery is also stenotic (top arrow) and the left subclavian artery is not visualized (second arrow from the top).
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Aortitis. Bilateral dilatation of the vertebral arteries. Occlusion of the right internal carotid artery (left arrow). Severe stenosis of the left internal carotid artery are shown. Note the moderate stenosis of the left external carotid artery at the bifurcation with dilated collateral circulation.
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Aortitis. This image reveals moderate stenosis of the external iliac artery at the bifurcation and occlusion of the right femoral artery.
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Aortitis. This image demonstrates leukocyte infiltration of the vasa vasorum of the aorta accompanies arteritis obliterans and ischemic necrosis of the media in a case of syphilitic aortitis.
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Aortitis. Granulomatous arteritis with thrombosis of a cerebral vessel may present as a neurologic defect with no obvious vascular disease by history or physical examination.
