Takayasu arteritis is a chronic vasculitis which affects the aorta and its major branches in young adults, predominantly females.
The disease follows a three phase clinical course:
(1) systemic symptoms (fever, myalgias, arthralgias, weight loss) develop, which resolve over several weeks, followed by arteritis, typically affecting the aortic arch and its major branches
(2) asymptomatic period
(3) vaso-occlusion with absence or decreased arterial pulses and bruits of the aorta or its major branches
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Criteria |
Comment |
|---|---|
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age of disease onset <= 40 years |
|
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claudication of extremities |
development and worsening of fatigue and discomfort in muscles of one or more extremities while in use, especially the upper extremities |
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decreased brachial artery pulse |
decreased pulsation in one or both brachial arteries |
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blood pressure difference > 10 mm Hg |
difference of > 10 mm Hg in systolic blood pressure between arms |
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bruit over subclavian artery or aorta |
bruit audible on auscultation over one or both subclavian arteries or abdominal aorta |
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arteriographic abnormalities |
narrowing, aneurysms or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia or similar causes; changes usually focal or segmental |
Additional findings:
(1) pulmonary artery involvement occurs in about half of the patients
(2) elevated erythrocyte sedimentation rate
(3) anemia
(4) hypergammaglobulinemia
(5) biopsy findings resemble temporal (giant cell) arteritis, with granulomatous inflammation of the arterial wall; eventually there is intimal hyperplasia, medial degeneration and adventitial fibrosis
(6) increased mortality (26% over 5 years) seen if ischemic retinopathy, severe hypertension, significant aortic valvular insufficiency, or an aneurysm of aorta or major blood artery occurs
Interpretation:
• A patient with 3 or more criteria can be diagnosed with Takayasu arteritis.
Performance:
• The sensitivity 90.5% and specificity 97.8%
Purpose: To diagnose Takayasu arteritis by the presence of certain findings.
Specialty: Immunology/Rheumatology
Objective: criteria for diagnosis
ICD-10: M31.4,
