2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

Abstract

BackgroundWe published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.ObjectiveTo update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010).MethodsThe Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) http://www.haecanada.com and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review.ResultsThis manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference.ConclusionsConsensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

“…Before attacks, many patients experience prodromal symptoms that can include tingling sensations or erythema marginatum, a nonpruritic and not raised rash (Figure 1)[4,21,24]. The patient's family history or frequency and severity of attacks do not predict the course of future attacks nor do they predict involvement of the airway during an attack[25]. …”

“…HAE should be suspected in patients presenting with any of the characteristic symptoms, especially in the presence of a positive family history[25]. Screening for genetic mutations is not generally performed for diagnostic reasons.…”

“…Instead, laboratory testing is indicated for patients with suspected HAE. Biochemical markers used for the diagnosis of HAE are serum complement factor 4 (C4), C1-esterase inhibitor antigen, functional C1-esterase inhibitor, and C1q antigenic protein[25]. The most cost-effective method to screen for HAE is measurement of C4 levels[2].…”

“…The most cost-effective method to screen for HAE is measurement of C4 levels[2]. If C4 is normal, as can occur in some patients between attacks, the measurement should be repeated during an acute attack[25]. Whether to obtain C1-esterase inhibitor antigen and functional C1-esterase inhibitor studies at the time of C4 collection or at a subsequent time can be based on the index of suspicion.…”

“…Low levels of C4, C1-esterase inhibitor antigenic protein, and functional C1-esterase inhibitor are consistent with a type I HAE diagnosis but should be confirmed via a second measurement. Low levels of C4 and functional C1-esterase inhibitor with normal or elevated C1-esterase inhibitor antigenic protein are indicative of type II HAE[25]. C1q antigenic protein is normal in HAE[25].…”