Angioedema disorder | C4* | C1-INH level | C1-INH function | C1q | Other tests |
Hereditary angioedema with C1-INH deficiency type I (HAE-C1-INH type I) | Low | Low | Low (usually <50% of normal) | Normal | Genetic testing (not needed for diagnosis) |
Hereditary angioedema with C1INH deficiency type II (HAE-C1-INH type II) | Low | Normal or elevated | Low (usually <50% of normal) | Normal | Genetic testing (not needed for diagnosis) |
Hereditary angioedema with factor XII gene mutations (HAE-FXII) | Normal | Normal | Normal | Normal | Mutations in gene for factor XII |
Hereditary angioedema with angiopoietin-1 gene mutations (HAE-ANGPT1) | Normal | Normal | Normal | Normal | Mutations in gene for angiopoietin-1 |
Hereditary angioedema with plasminogen gene mutations (HAE-PLG) | Normal | Normal | Normal | Normal | Mutations in gene for plasminogen |
Hereditary angioedema with kininogen-1 gene mutations (HAE-KNG1) | Normal | Normal | Normal | Normal | Mutations in gene for kininogen-1 |
Hereditary angioedema of unknown origin (HAE-U) | Normal | Normal | Normal | Normal | |
Acquired angioedema with C1-INH deficiency (AAE-C1-INH) | Low | Normal or low | Low (usually <50% of normal) | Normal or low¶ | Anti-C1-INH antibodies (not needed for diagnosis) |
Idiopathic acquired angioedema (histaminergic or nonhistaminergic) (AAE-IH or AAE-InH) | Normal | Normal | Normal | Normal | |
ACE inhibitor-associated angioedema (AAE-ACEI) | Normal | Normal | Normal | Normal |
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