Table 1.
C1Inh-HAE | C1Inh-AAE | nC1Inh-HAE | ACEi-AAE | ||
---|---|---|---|---|---|
HAE Type I | HAE Type II | ||||
C1Inh function | <50 % | < 50% | <50% | Normal | Normal |
C1Inh | <50% | Normal | <50% | Normal | Normal |
C4 | Low | Low | Low | Normal | Normal |
C1q | Normal | Normal | Low (70% of cases) | Normal | Normal |
Anti-C1Inh Ab | Present (50% of cases) | ||||
Mutation | SERPING1 | SERPING1 | No | FXII, PLG, ANGPT1, KNG1 | No |
Abbreviations: AE, angioedema; C1Inh-HAE, hereditary AE with C1Inhibitor deficiency; C1Inh, C1 inhibitor, C1Inh-AAE, acquired AE with C1-inhibitor deficiency; nC1Inh-HAE, acquired angioedema with normal C1-inhibitor; ACEi-AAE, angiotensin-converting enzyme inhibitor-induced AE; Ab, antibody.