Pathology · Vascular Pathology (Atherosclerosis, Vasculitis, Aneurysm)

A 28-year-old woman of East Asian descent presents with unequal upper limb blood pressures, absent radial pulse on the left, and elevated ESR. Aortography shows stenosis of the left subclavian and left common carotid arteries with a 'skip lesion' pattern. ANCA and ANA are negative. Which pathological type of arteritis best explains this?

  • A Takayasu arteritis — granulomatous large-vessel vasculitis predominantly in young Asian women, affecting the aorta and its branches
  • B Giant cell (temporal) arteritis — affects medium/large arteries in elderly patients >50 years
  • C Polyarteritis nodosa — necrotizing medium-vessel vasculitis with renal and visceral vessel involvement, ANCA negative
  • D Kawasaki disease — mucocutaneous lymph node syndrome in children with coronary aneurysm risk
Correct answer: A. Takayasu arteritis — granulomatous large-vessel vasculitis predominantly in young Asian women, affecting the aorta and its branches

Explanation

Takayasu arteritis ('pulseless disease') is a granulomatous panarteritis of the aorta and its major branches, predominantly affecting young women of Asian descent. The inflammatory infiltrate includes CD4+ and CD8+ T cells, NK cells, and macrophages/giant cells primarily in the media and adventitia. Elastic fiber destruction and intimal proliferation cause stenosis ('stenotic phase') or aneurysm formation. Serum IL-6 and IL-17 are elevated, and IL-6 pathway (tocilizumab) is now a guideline-recommended steroid-sparing therapy. Giant cell arteritis affects patients >50 years. PAN is a medium-vessel necrotizing vasculitis. Kawasaki is a pediatric disease.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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