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Chapter 22: Hereditary and acquired angioedema.

Mary S Georgy, Jacqueline A Pongracic

Allergy and Asthma Proceedings

|July 17, 2012

Summary

Hereditary angioedema (HAE) and acquired angioedema (AAE) involve C1 esterase inhibitor (C1-INH) deficiency. Diagnostic tests include C4 and C1q levels, with specific therapies available for these potentially life-threatening conditions.

Area of Science:

Immunology
Genetics
Pharmacology

Background:

Hereditary angioedema (HAE) and acquired angioedema (AAE) are rare, potentially life-threatening conditions characterized by C1 esterase inhibitor (C1-INH) deficiency or dysfunction.
Both HAE and AAE can manifest with low or absent complement component C4 levels, complicating diagnosis.
Understanding the distinct types of HAE (Type 1, Type 2, and Type 3) and their underlying mechanisms is crucial for effective management.

Purpose of the Study:

To elucidate the diagnostic markers and differentiations between HAE and AAE.
To review the classification and underlying pathophysiology of different HAE subtypes.
To highlight current and emerging therapeutic strategies for managing angioedema episodes.

Main Methods:

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Review of diagnostic criteria and laboratory tests, including C4 and C1q protein levels.

Classification of HAE into types based on C1-INH levels and functional activity.

Examination of genetic factors, such as Factor XII mutations in Type 3 HAE.

Analysis of therapeutic interventions, including danazol and disease-specific treatments.

Main Results:

Complement component C4 is a screening test for both HAE and AAE, while C1q levels help differentiate between them (normal in HAE, low in AAE).
HAE Type 1 involves decreased C1-INH production, Type 2 involves dysfunctional C1-INH, and Type 3 is rare, estrogen-dependent, and linked to Factor XII mutations.
Anabolic steroid danazol can increase C4 levels and reduce angioedema frequency but has adverse effects.
Disease-specific therapies include C1-INH concentrate, kallikrein inhibitor ecallantide, and bradykinin B2-receptor antagonist icatibant.

Conclusions:

Accurate differentiation between HAE and AAE is critical for appropriate treatment selection.
Advances in understanding HAE subtypes and their genetic underpinnings are improving diagnostic accuracy.
Targeted therapies offer effective management options for acute angioedema attacks, improving patient outcomes.