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Two international working groups have formed to date and each produced
a consensus document presenting the views of the experts from the
centres involved. These documents focus on how HAE should be diagnosed
and which treatment options used in each case. This has not resulted in
a standard procedure, as there are some differences between the two,
particularly in terms of the options selected for treatment.
Both documents were produced at a time when drugs that are now or soon might be in the approval process, such as recombinant C1 inhibitor (Pharming), plasma derived C1 inhibitor (Lev), kallikrein inhibitor DX-88 (Dyax Inc.) or the bradykinin B2 receptor antagonist Icatibant (Jerini AG), were not available. It should be assumed that approval of these substances, some of which look very promising, will change the treatment algorithms again.
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Consensus documents
Consensus documents on the treatment of HAE
Canadian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema
Bowen T; Cicardi M; Bork K; Zuraw B; Frank M, et al.
Annals of allergy, asthma & immunology 2007; 100:30-40
Abstract
Background: We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) in 2004.Objective: To ensure that this consensus remains current.
Methods: In collaboration with the Canadian Network of Rare Blood Disorder Organizations, we held the second Canadian Consensus discussion with our international colleagues in Toronto, Ontario, on February 3, 2006, and reviewed its content at the Fifth C1 Inhibitor Deficiency Workshop in Budapest on June 2, 2007. Papers were presented by international investigators, and this consensus algorithm approach resulted.
Results: This consensus algorithm outlines the approach recommended for the diagnosis, therapy, and management of HAE, which was agreed on by the authors of this report. This document is only a consensus algorithm approach and continues to require validation. As such, participants agreed to make this a living 2007 algorithm, a work in progress, and to review its content at future international HAE meetings.
Conclusions: There is a paucity of double-blind, placebo-controlled trials on the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Controlled trials currently under way will provide further insight into the management of HAE. As with our Canadian 2003 Consensus, this 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of HAE was formed through the meeting and agreement of patient care professionals along with patient group representatives and individual patients.
Full paper vie Annals of Allergy, Asthma and Immunology (subscribers only)
C1 inhibitor deficiency: consensus document
Gompels MM, Lock RJ, Abinun M, Bethune CA, Davies G, et al.
Clin. and Exp. Immunol. 2005; 139:379-394
Abstract
We present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein.The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants.
Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid.
There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist.
Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification.
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