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Comparison of HAE and AAE.
| Age of onset | Family history | Complement levels | ||||
|---|---|---|---|---|---|---|
| C1q | C4 | C1 inh level | C1 inh function | |||
|
HAE Type 1 Type 2 |
Early |
Yes* |
Normal Normal |
Low Low |
Low Normal/elevated |
Low Low |
| AAE | Late | No | Low | Low | Normal or low | Low |
*In approximately 25% of patients, no family history is identified; the disorder results from spontaneous mutation of the C1 inhibitor gene.
C1 inh: C1 inhibitor
