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Currently available treatments and future treatment options for hereditary angioedema

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Summary

Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by recurrent skin swellings, abdominal pain attacks, and — more rarely — potentially life-threatening laryngeal attacks. Thus, HAE-C1-INH may be associated with a significant morbidity and mortality. Over the last years, the field of research in the pathogenesis of HAE-C1-INH has greatly expanded. This has led to new clinical trials with new therapeutic options. Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available for treating acute attacks include plasma-derived C1-INH concentrates, a bradykinin B2 receptor antagonist, and a recombinant human C1-INH. In the USA, a plasma kallikrein inhibitor has additionally been approved. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics. Plasma-derived C1-INH and a bradykinin B2-receptor antagonist are approved for self-administration at home. The number of management options for HAE-C1-INH has increased considerably within the last few years, thus helping to alleviate the burden of disease.

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Abbreviations

BW:

Body weight

B2R:

Bradykinin B2-receptor

C1-INH:

C1 inhibitor

HAE-C1-INH:

Hereditary angioedema due to C1-INH deficiency

HAE:

Hereditary angioedema

HAEnCI:

HAE with normal C1-INH

KKS:

Kallikrein-kinin system

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Authors and Affiliations

  1. Hautklinik, Universitätsmedizin Mainz Johannes-Gutenberg-Universität, Langenbeckstraße 1, 55131, Mainz, Germany

    Konrad Bork

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Correspondence to Konrad Bork.

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Cite this as

Bork K. Currently available treatments and future treatment options for hereditary angioedema. Allergo J Int 2015;24:122–8 DOI: 10.1007/s40629-015-0062-4

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Bork, K. Currently available treatments and future treatment options for hereditary angioedema. Allergo J 24, 32–38 (2015). https://doi.org/10.1007/s15007-015-0852-5

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  • DOI: https://doi.org/10.1007/s15007-015-0852-5

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