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polyarteritis nodosa (PAN)

see also:

introduction

  • rare disease
  • incidence of about 3-4.5 cases per 100,000 population annually
  • males 2x females
  • esp. ages 45-65yrs
  • systemic vasculitis involving medium to small arteries
  • most commonly affects skin, joints, peripheral nerves, the gut, and the kidney
  • nodular skin lesions, neuropathy, and hypertension
  • hepatitis B virus associated vasculitis almost always takes the form of PAN, and usually has onset in 1st 6 months of infection
    • HBV accounted for 30% of PAN but since widespread vaccination, it now only accounts for < 8% of cases
  • many other infections and inflammatory diseases have been associated with possibly causing PAN including hepatitis C virus
  • untreated, the 5-year survival rate of PAN is 13%
  • corticosteroids treatment improves the 5-year survival rate to 50-60%
  • adding immunosuppressants such as cyclophosphamide, the 5-year survival rate may increase to greater than 80%

diagnostic criteria

  • at least 3 of the 10 ACR criteria should be present when radiographic or pathological diagnosis of vasculitis is made:
    • weight loss of 4 kg or more
    • livedo reticularis
    • testicular pain/tenderness
    • myalgia or leg weakness/tenderness
    • mononeuropathy or polyneuropathy
    • diastolic blood pressure > 90 mm/Hg
    • levated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
    • presence of hepatitis B surface antigen or antibody in serum
    • arteriogram demonstrating aneurysms or occlusions of the visceral arteries
    • biopsy of small- or medium-sized artery containing polymorphonuclear neutrophils

diagnostic workup

childhood systemic polyarteritis nodosa

  • small and mid-size artery necrotizing vasculitis on biopsy with at least 2 of:
    • skin involvement (eg, livedo reticularis, tender subcutaneous nodules, other vasculitic lesions)
    • myalgia
    • systemic hypertension
    • mononeuropathy or polyneuropathy
    • abnormal urinalysis and/or impaired renal function (GFR < 50% normal for age)
    • testicular pain or tenderness
    • signs or symptoms suggesting vasculitis of any other major organ system (GI, cardiac, pulmonary, CNS)

childhood cutaneous polyarteritis nodosa

  • presence of subcutaneous nodular, painful, nonpurpuric lesions with or without livedo reticularis and absence of systemic involvement
  • associated with streptococcus / streptococcal infections infection in 40%
pan.txt · Last modified: 2013/08/14 21:55 (external edit)