Figure 1. Recurrent Angioedema – Diagnostic Algorithm
Recurrent
Angioedema
ACE-I/ARB-
associated AE
Not likely HAE or acquired
C1INH deficiency; exclude
drug cause and consider
idiopathic
NO
YES
YES
Is patient
taking an ACE-I
or ARB?
NO
Stop ACE-I/
ARB. Does AE
resolve?
LOW
Consider
HAE with
normal C1INH
NORMAL
YES
Measure C4
level
Not HAE;
consider
idiopathic AE
NORMAL
Measure
C1INH
antigen
NORMAL
NO
Is there a
familial history
of AE?
LOW
NORMAL
Measure C1q
antigen
Type I HAE
Type II HAE
Measure
C1INH
function
LOW
Acquired C1INH
deficiency
AE & Urticaria
AE Only
LOW
Diagnosis