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Diagnostic and therapeutic management of hereditary angioedema due to C1-inhibitor deficiency: the Italian experience.
Curr Opin Allergy Clin Immunol 2015; 15(4):383-91CO

Abstract

PURPOSE OF REVIEW

Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare disease, with a reported prevalence of about 1 : 50 000. C1-INH-HAE causes disabling symptoms, which may be life-threatening if swelling affects upper airways. Diagnostic procedures are now well established and the role of bradykinin as the main mediator of plasma outflow eliciting angioedema formation has been clearly elucidated.

RECENT FINDINGS

Increased understanding of the pathogenesis of C1-INH-HAE allowed in recent years the development of new drugs targeted to inhibit bradykinin synthesis (Ecallantide) or activity (Icatibant). At the same time, a recombinant C1-INH concentrate (Ruconest) was produced from the milk of transgenic rabbits and two plasma-derived C1-INHs (Berinert, Cinryze) underwent controlled trials to obtain marketing authorization. In 2012, an Italian network for C1-INH-HAE (ITACA) was established by physicians of 17 HAE reference centres to collect data from Italian patients and to homogenize and improve the diagnostic and therapeutic approach to the disease.

SUMMARY

Although there is a widespread agreement on therapeutic goals and treatment of C1-INH-HAE acute attacks, different approaches to prophylaxis are still present among HAE experts. The clinical experience of ITACA on a large population of C1-INH-HAE patients followed for several years may help in identifying the most effective strategies for the management of the disease.

Authors+Show Affiliations

Dipartimento di Medicina, Università degli Studi di Padova, Padova, Italy.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

26106828

Citation

Cancian, Mauro, and Italian network for C1-INH-HAE (ITACA). "Diagnostic and Therapeutic Management of Hereditary Angioedema Due to C1-inhibitor Deficiency: the Italian Experience." Current Opinion in Allergy and Clinical Immunology, vol. 15, no. 4, 2015, pp. 383-91.
Cancian M, Italian network for C1-INH-HAE (ITACA). Diagnostic and therapeutic management of hereditary angioedema due to C1-inhibitor deficiency: the Italian experience. Curr Opin Allergy Clin Immunol. 2015;15(4):383-91.
Cancian, M. (2015). Diagnostic and therapeutic management of hereditary angioedema due to C1-inhibitor deficiency: the Italian experience. Current Opinion in Allergy and Clinical Immunology, 15(4), pp. 383-91. doi:10.1097/ACI.0000000000000186.
Cancian M, Italian network for C1-INH-HAE (ITACA). Diagnostic and Therapeutic Management of Hereditary Angioedema Due to C1-inhibitor Deficiency: the Italian Experience. Curr Opin Allergy Clin Immunol. 2015;15(4):383-91. PubMed PMID: 26106828.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Diagnostic and therapeutic management of hereditary angioedema due to C1-inhibitor deficiency: the Italian experience. AU - Cancian,Mauro, AU - ,, PY - 2015/6/25/entrez PY - 2015/6/25/pubmed PY - 2016/3/19/medline SP - 383 EP - 91 JF - Current opinion in allergy and clinical immunology JO - Curr Opin Allergy Clin Immunol VL - 15 IS - 4 N2 - PURPOSE OF REVIEW: Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare disease, with a reported prevalence of about 1 : 50 000. C1-INH-HAE causes disabling symptoms, which may be life-threatening if swelling affects upper airways. Diagnostic procedures are now well established and the role of bradykinin as the main mediator of plasma outflow eliciting angioedema formation has been clearly elucidated. RECENT FINDINGS: Increased understanding of the pathogenesis of C1-INH-HAE allowed in recent years the development of new drugs targeted to inhibit bradykinin synthesis (Ecallantide) or activity (Icatibant). At the same time, a recombinant C1-INH concentrate (Ruconest) was produced from the milk of transgenic rabbits and two plasma-derived C1-INHs (Berinert, Cinryze) underwent controlled trials to obtain marketing authorization. In 2012, an Italian network for C1-INH-HAE (ITACA) was established by physicians of 17 HAE reference centres to collect data from Italian patients and to homogenize and improve the diagnostic and therapeutic approach to the disease. SUMMARY: Although there is a widespread agreement on therapeutic goals and treatment of C1-INH-HAE acute attacks, different approaches to prophylaxis are still present among HAE experts. The clinical experience of ITACA on a large population of C1-INH-HAE patients followed for several years may help in identifying the most effective strategies for the management of the disease. SN - 1473-6322 UR - https://www.unboundmedicine.com/medline/citation/26106828/Diagnostic_and_therapeutic_management_of_hereditary_angioedema_due_to_C1_inhibitor_deficiency:_the_Italian_experience_ L2 - http://Insights.ovid.com/pubmed?pmid=26106828 DB - PRIME DP - Unbound Medicine ER -