Polyarteritis nodosa (PAN):
• sometimes, historically, used as generic name for this group of vasculitis conditions
• widespread necrotising inflammatory arteritis can lead to life threatening multisystem disease.
• M 2-3x>F. Annual incidence of 5-10/1 000 000. Onset usually 40-60yrs.
• affects small and medium size arteries of skin, kidney, peripheral nerves, muscle and stomach – usually young men with no underlying disease
• often abrupt onset with fever, weight loss, joint pain (50-75%; similar to rheumatoid arthritis), skin lesions – palpable purpura/nodules (along course of artery), ulcerations and infarctions (25-50%), neurologic symptoms (includes a symmetric sensory and motor polyneuropathy), hypertension, renal disease (25%-80%)
• prognosis depends on extent of visceral and CNS involvement – but generally poor. 5 year survival rate is about 50%
• if extent of disease is limited corticosteroids
• if extensive visceral involvement cytotoxic agent
Comments are closed.