Pathophysiology
1) inflammation of large arteries (especially aorta and branches) 2) type 1 – involvement of the aortic arch and branches 3) type 2 – involvement of descending thoracic and abdominal aorta and branches 4) type 3 – involvement of both arch and descending aorta and branches 5) other vessels commonly involved – retinal, CNS, and pulmonary arteries
Signs and Symptoms
1) dizziness/syncope 2) visual problems 3) dyspnea 4) intermittent claudication 5) loss of pulses 6) hypertension 7) arterial bruits 8) fever/night sweats 9) weight loss 10) malaise 11) arthralgias 12) anorexia 13) pain over involved vessels
Characteristic Test Findings
Laboratory – 1) increased serum renin (owing to renal artery stenosis) 2) mild anemia 3) increased sed rate 4) increased serum immunoglobulin levels Radiology – 5) stenosis and occlusion of affected arteries and aneurysms in aortic arch and distal aorta on arteriogram
Histology/Gross Pathology
1) thickened, focal, raised, plaques in arteries 2) panarteritis of walls with mononuclear cells, neutrophils, and Langerhans’ cells 3) intimal hyperplasia
Associated Conditions
1) stroke 2) relapsing polychondritis
Inheritance/Epidemiology
1) 90% of patients are women 2) onset usually before age 30 years 3) most patients are Asian 4) highly variable course with fulminant disease to spontaneous remissions
Treatment
1) early in course – corticosteroids or immunosuppression drugs (especially methotrexate) 2) late treatment – requires surgical bypass or atherectomy
Tips for USMLE
1) almost always affects the subclavian arteries 2) if a 23 year old Japanese woman develops pain over her right carotid artery, a blood pressure of 140/60 in her left arm and 80/40 in her right arm and no detectable pulse at her right radial artery, think Takayasu’s
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