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Table 2 Comparison of HAE and AAE [7]

From: Angioedema

 

Family history

Complement levels/laboratory findings

C4

C1-INH antigen

C1-INH function

C1q

HAE-1

HAE-2

Yes*

Normal or ↑

Normal

HAE-nC1-INH

Yes

Normal

Normal

Normal

Normal

AAE

No

Normal or ↓

Low

  1. *In approximately 25% of patients, no family history is identified; the disorder results from spontaneous mutation of the C1 inhibitor gene
  2. C1q level testing may be difficult to obtain in Canada
  3. Adapted from: Betschel et al. 2019 [7]