Definition
• Arteritis involving large and medium-sized arteries; preferentially involving the aorta and its main branches
Clinical features
Epidemiology
• Up to 3.3 cases per million people (most common in Asia)
• Young females (male:female ratio, 1:3)
• Patients usually range from 14 to 66 years of age; mean age at diagnosis, 25 years
• Associated with HLA-DR4 haplotype
Presentation
• Hypertension is most common presenting condition (due to either coarctation of the aorta or renal arterial stenosis)
• Fever, arthritis, myalgia, skin nodules
• Weak or absent pulses in upper extremities due to stenosis of aorta or proximal great vessels (“pulseless disease”)
• Cerebrovascular symptoms may also be seen
• Elevated erythrocyte sedimentation rate (ESR)
• Diagnosis requires fulfilling three or more of the following diagnostic criteria (from Arend WP, Michel BA, Bloch DA, et al: The American College of Rheumatology 1990 criteria for the classification of Takayasu’s arteritis, Arthritis Rheum 33:1129-1134, 1990):
• Age younger than 40 years
• Claudication of extremities
• Decreased brachial artery pulse
• Systolic blood pressure difference of 10 mm Hg between upper limbs
• Bruit over aorta or subclavian arteries
• Abnormal arteriogram demonstrating stenosis of aorta or proximal great vessels
Prognosis and treatment
• Chronically remitting and relapsing disease with poorly defined long-term clinical outcome: some patients have aggressive disease, whereas others show stable disease with long-term survival
• Long-term glucocorticoid therapy
• Cardiac failure is the most common cause of death
Pathology
Histology
• Early lesion: granulomatous vasculitis involving media and adventitia; intimal thickening leads to arterial stenosis
• Later lesion: fibrosis of arterial wall
Immunopathology/special stains
• Not contributory
Main differential diagnosis
• Giant cell arteritis: associated with polymyalgia rheumatica; affects older patients
Fig 1 Takayasu arteritis. Necrotizing granulomatous inflammation involving the aortic wall in this 16-year-old female with aortic aneurysm (low power).
Fig 2 Takayasu arteritis. Poorly formed granulomas with basophilic necrosis are seen with medium power.
Fig 3 Takayasu arteritis. Multinucleated giant cells and neutrophils are present in the aortic granuloma.