Hereditary and acquired angioedema

  • Facts:
    Pathology of both forms involve C1 inhibitor deficiency, dysfunction, or destruction

    Hereditary
    Presents in late childhood, usually follows an infection, dental procedure, or trauma
    Normal C1q levels

    Acquired
    MCC is ACEI use
    Depressed C1q levels

    C1 esterase inhibitor deficiency leads to elevated levels of C2b and bradykinin (edema-producing factors)
  • Diagnosis:
    C1 esterase inhibitor deficiency
    Depressed C4 levels in both forms
  • Treatment:
    Hereditary angioedema
    C1 inhibitor concentrate, Kallikrein inhiitor, FFP
  • See Also:
    Angioedema



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September 8th 2010