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تعداد آیتم قابل مشاهده باقیمانده: 4

Comparison of complement studies in angioedema disorders

Comparison of complement studies in angioedema disorders
Angioedema disorder C4* C1INH level C1INH function C1q Other tests
Hereditary angioedema with C1INH deficiency type 1
(HAE-C1INH type 1)		 Low Low Low
(usually <50% of normal)		 Normal Genetic testing
(not needed for diagnosis)
Hereditary angioedema with C1INH deficiency type 2
(HAE-C1INH type 2)		 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 C1INH deficiency
(AAE-C1INH)		 Low Normal or low Low
(usually <50% of normal)		 Normal or low Anti-C1INH 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-C1INH and C1INH-HAE are both used for hereditary angioedema in the literature.

AAE: acquired angioedema; ACEI: angiotensin-converting enzyme inhibitor; C1INH: C1 inhibitor; HAE: hereditary angioedema.

* In HAE-C1INH types 1 and 2, 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.

Graphic 83098 Version 11.0

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