1/ New guidelines from the @US_HAEA Advisory Board are now available @JACIInPractice. We provide an update on classification, diagnosis, on-demand/prophylactic tx with special considerations for women/children along with guidance on development of a comprehensive management plan. 
2/#HAE can be broadly divided into #HAE due to C1INH deficiency (type 1 and type 2) and HAE with normal labs (4 mutations now identified - factor XII, plasminogen,
angiopoetin-1, and kininogen). Early diagnosis is critical. If you suspect send C4, C1INH level and function.
angiopoetin-1, and kininogen). Early diagnosis is critical. If you suspect send C4, C1INH level and function.
3/All patients with #HAE must have ready access to effective on-demand medication and trained in self-administration. Encourage on-demand treatment of all #HAE attacks. Four FDA approved on-demand treatments available (ecallantide, icatibant, pdC1INH, or rhC1INH)
4/Long-term prophylactic treatment of HAE due to C1INH deficiency should include first-line treatments (IV or SC C1INH or #lanadelumab). Oral #kallikrein inhibitor (#berotralstat) FDA approved Dec 2020! Decision on long-term prophylaxis should reflect needs of individual patient.
5/Management plans should be individualized to lessen the burden of illness and aim to provide patients with #HAE with a normal quality of life. @mghaifellows @mghallergy jaci-inpractice.org/action/showPdf…
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