22- LARGE CELL VASCULITIS(aorta & its major branches)

• Polymyalgia (PMR)


  Systemic illness affecting patients older than 50 years
• Giant cell arteritis (GCA)


  Systemic illness affecting patients older than 50 years
• Polymyalgia (PMR) and giant cell arteritis (GCA)


  Both are associated with the finding of GCA on temporal artery biopsy
• Polymyalgia Rheumatica


  • Abrupt onset of stiffness and intense pain in the muscles of the neck and shoulder, and hips and lumbar spine
  • Constitutional symptoms, with malaise, fever, weight loss and anorexia
  • Arteritic involvement by inflammation in the superficial temporal arteries à headache, tenderness over the scalp (combing the hair may be painful) and claudication of the jaw when eating
• Giant cell arteritis


  • Affecting the vertebrobasilar and sometimes the carotid circulation may result in stroke
  • Most devastating complication of GCA: sudden loss of vision (may be permanent) due to involvement of the ophthalmic artery
• May have symptoms limited to PMR or to GCA alone, or manifestations of both
• Investigations


  • Very high ESR (around 100 mm/h) and CRP
  • Normochromic, normocytic anemia
  • Temporal artery biopsy is performed if GCA is suspected and should be performed before or within a week of starting corticosteroids
• Management


  1. Treatment is started based on a clinical diagnosis

  2. Corticosteroids (high dose prednisolone)

  3. Prophylaxis against steroid-induced osteoporosis should be given
• 2.! Takayasu’s arteritis

  • Epidemiology: rare except in Japan.

  • Pathophysiology: Vasculitis involving the aortic arch and other major arteries

  • Symptoms: Hypertension, absent peripheral pulses, strokes and cardiac failure.

  • Treatment: corticosteroids.