Vascular

Giant cell arteritis

PATHO

  • Autoimmune vasculitis ➔ chronic inflammation of medium & large arteries (carotid and aorta)

  • Inflammation ➔ narrowing ➔ ↓ blood flow ➔ ischemia

SSX

  • Constitutional: Fever, night sweats, malaise, myalgia (shoulder and hip muscle pain)

    • 50% of pts with GCA have polymyalgia rheumatica

  • Cranial:

    • Temporal artery (MC) ➔ hardened and tender, headache over temple

      • Jaw claudication (chewing ➔ ↑ demand ➔ ischemia ➔ pain)

    • Amaurosis fugax (ischemic optic neuropathy) and other vision changes

  • Large vessel (LC) ➔ angina, ABD pain, limb claudication

DX

  • ↑ ESR/CRP

  • Duplex US of temporal artery shows edema and thickened vessel walls (halo) and stenotic, noncompressible artery

    • Occluded/inflamed arteries are not compressible (like veins)

  • Temporal biopsy (definitive dx) shows mononuclear infiltration of vessel walls with formation of giant cells

TX

  • DON'T DELAY TX FOR DEFINITIVE DX

    • Delay can ➔ irreversible vision loss

  • ↑ clinical suspicion is enough to start high dose (IV) glucocorticoids

Thromboangiitis obliterans (Buerger disease)

PATHO

  • Inflammatory vaso-occlusive disease of small & medium sized arteries & veins

  • ♂> ♀ (3:1), ↑with smoking

  • Inflammation of tunica intimamicroabscess (endarteritis) ➔ thrombi within lumen

    • Most of the vessel wall (media/lamina) is usually spared

  • Affects distal arteries of upper and lower extremities

SSX

  • Initial presentation:

    1. Intermittent claudication

    2. Raynaud phenomenon

    3. Superficial thrombophlebitis (usually migratory)

  • Critical limb ischemia ➔ pain at rest ➔ gangrene

  • Brachial and politeal pulses normal; weak distally

DX

  • ESR/CRP normal

  • ABI usually decreased

  • Abnormal Allen's test

  • Arteriography > Doppler US

    • Shows non-atherosclerotic, segmental lesions (source of occlusion) with corkscrew-shaped collateral vessels

  • Biopsy is definitive but rarely needed

  • Corkscrew collaterals on arteriography are not pathognomonic for thromboangiitis obliterans

    • Seen in PAD that affects small & medium-sized arteries

TX

  • Stop smoking, wound care

  • Iloprost (prostacyclin analog ➔ arterial dilation) , dihydropyridine CCB (nifedipine, amlodipine)