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    Subjects/Medicine/Vasculitis — Clinical
    Vasculitis — Clinical
    medium
    stethoscope Medicine

    A 58-year-old man with a 3-month history of progressive constitutional symptoms, bilateral lower limb claudication, and pulseless femoral arteries is found to have elevated inflammatory markers. Angiography reveals smooth, tapered stenosis of the abdominal aorta and its branches. Which of the following is NOT a typical feature of Takayasu arteritis?

    A. Involvement of the pulmonary artery with potential right ventricular hypertrophy
    B. Acute phase characterized by granulomatous inflammation with giant cells on histology
    C. Frequent association with antineutrophil cytoplasmic antibody (ANCA) positivity
    D. Predilection for women in the reproductive age group (15–40 years)

    Explanation

    ## Key Distinguishing Feature of Takayasu Arteritis **Key Point:** Takayasu arteritis (TA) is a large-vessel vasculitis that is characteristically ANCA-negative, despite being a systemic inflammatory condition. ANCA positivity is NOT a typical feature and, when present, should prompt consideration of alternative diagnoses. ### Typical Features of Takayasu Arteritis | Feature | Status in TA | |---------|-------------| | **Age of onset** | Young women (15–40 years, 80% female) | | **Vessel involvement** | Large elastic arteries: aorta, pulmonary arteries, coronary ostia | | **Histology** | Granulomatous inflammation with giant cells, intimal proliferation | | **ANCA serology** | Negative (ANCA-negative vasculitis) | | **Pulmonary involvement** | Common (pulmonary artery stenosis, pulmonary hypertension) | | **Aortic involvement** | Aortic root dilatation, aortic regurgitation, aortic stenosis | **High-Yield:** TA is one of the few large-vessel vasculitides that is **ANCA-negative**. This is a critical distinguishing point from ANCA-associated vasculitides (AAV) such as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). ### Clinical Pearl **Clinical Pearl:** The patient's presentation of aortic branch stenosis with claudication and pulseless extremities is classic for TA. The smooth, tapered stenosis pattern (rather than irregular atherosclerotic lesions) and young age support the diagnosis. ANCA positivity would be atypical and unusual in TA. ### Mnemonic for Large-Vessel Vasculitis **Mnemonic:** **TACO** = **T**akayasu (ANCA-negative), **A**orta-involved, **C**oronary risk, **O**lder women (actually young women for TA, but the point is large vessels). Remember: Takayasu = **ANCA-negative**. [cite:Harrison 21e Ch 319]

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