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An evidence based therapeutic approach to hereditary and acquired angioedema.
Curr Opin Allergy Clin Immunol 2014; 14(4):354-62CO

Abstract

PURPOSE OF REVIEW

Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH), HAE with normal C1-INH, and acquired angioedema due to C1-INH deficiency are rare but important diseases that can be associated with significant morbidity and mortality. Research into the pathogenesis of angioedema has expanded greatly and has led to new clinical trials with novel therapeutic agents and strategies.

RECENT FINDINGS

Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available in Europe for treating acute attacks include plasma-derived C1-INH concentrates, a bradykinin B2 receptor (B2R) antagonist, and a recombinant human C1-INH. In the USA, a plasma-derived C1-INH concentrate, a bradykinin B2R antagonist, and a plasma kallikrein inhibitor have been approved for the treatment of acute HAE-C1-INH attacks. C1-INH concentrates and attenuated androgens are used for short-term prophylactic treatment. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics. Plasma-derived C1-INH and a bradykinin B2R antagonist are approved for self-administration at home.

SUMMARY

The number of management options for HAE-C1-INH and similar conditions has increased considerably within the last few years, thus helping to alleviate the burden of these rare diseases.

Authors+Show Affiliations

Department of Dermatology, Johannes Gutenberg University, Mainz, Germany.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

24983294

Citation

Bork, Konrad. "An Evidence Based Therapeutic Approach to Hereditary and Acquired Angioedema." Current Opinion in Allergy and Clinical Immunology, vol. 14, no. 4, 2014, pp. 354-62.
Bork K. An evidence based therapeutic approach to hereditary and acquired angioedema. Curr Opin Allergy Clin Immunol. 2014;14(4):354-62.
Bork, K. (2014). An evidence based therapeutic approach to hereditary and acquired angioedema. Current Opinion in Allergy and Clinical Immunology, 14(4), pp. 354-62. doi:10.1097/ACI.0000000000000082.
Bork K. An Evidence Based Therapeutic Approach to Hereditary and Acquired Angioedema. Curr Opin Allergy Clin Immunol. 2014;14(4):354-62. PubMed PMID: 24983294.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An evidence based therapeutic approach to hereditary and acquired angioedema. A1 - Bork,Konrad, PY - 2014/7/2/entrez PY - 2014/7/2/pubmed PY - 2015/2/13/medline SP - 354 EP - 62 JF - Current opinion in allergy and clinical immunology JO - Curr Opin Allergy Clin Immunol VL - 14 IS - 4 N2 - PURPOSE OF REVIEW: Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH), HAE with normal C1-INH, and acquired angioedema due to C1-INH deficiency are rare but important diseases that can be associated with significant morbidity and mortality. Research into the pathogenesis of angioedema has expanded greatly and has led to new clinical trials with novel therapeutic agents and strategies. RECENT FINDINGS: Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available in Europe for treating acute attacks include plasma-derived C1-INH concentrates, a bradykinin B2 receptor (B2R) antagonist, and a recombinant human C1-INH. In the USA, a plasma-derived C1-INH concentrate, a bradykinin B2R antagonist, and a plasma kallikrein inhibitor have been approved for the treatment of acute HAE-C1-INH attacks. C1-INH concentrates and attenuated androgens are used for short-term prophylactic treatment. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics. Plasma-derived C1-INH and a bradykinin B2R antagonist are approved for self-administration at home. SUMMARY: The number of management options for HAE-C1-INH and similar conditions has increased considerably within the last few years, thus helping to alleviate the burden of these rare diseases. SN - 1473-6322 UR - https://www.unboundmedicine.com/medline/citation/24983294/An_evidence_based_therapeutic_approach_to_hereditary_and_acquired_angioedema_ L2 - http://Insights.ovid.com/pubmed?pmid=24983294 DB - PRIME DP - Unbound Medicine ER -