investigation

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During an acute attack there are:

  • elevated plasma levels of activated C1
  • decreased levels of C2 and C4

In between attacks

  • decreased levels of C4 (rarely levels may be normal between attacks)
  • normal C1 and C3 levels
  • antigenic and functional C1-inhibitor levels differentiate type 1 hereditary angioedema from type 2
    • in type 1 - both antigenic and functional C1-inhibitor levels are low
    • in type 2 - antigenic C1-inhibitor levels are normal but functional C1-inhibitor activity is low (1)

Reference:

Last reviewed 01/2018

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