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Comparison of complement studies in angioedema disorders

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

Terminology for angioedema disorders is evolving, and the abbreviations HAE-C1-INH and C1INH-HAE are both used for hereditary angioedema in the literature.

C1-INH: C1 inhibitor; HAE: hereditary angioedema; AAE: acquired angioedema; ACEI: angiotensin-converting enzyme inhibitor.
* In HAE-C1-INH types I and II, C4 is always low during an attack (with one published exception) and are chronically low in the majority of patients.
¶ There are rare forms of acquired angioedema in which C1q levels are normal. Refer to the UpToDate topics on acquired C1 inhibitor deficiency for details.

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