| Literature DB >> 35006617 |
Marcus Maurer1,2, Markus Magerl1,2, Stephen Betschel3, Werner Aberer4, Ignacio J Ansotegui5, Emel Aygören-Pürsün6, Aleena Banerji7, Noémi-Anna Bara8, Isabelle Boccon-Gibod9, Konrad Bork10, Laurence Bouillet9, Henrik Balle Boysen11, Nicholas Brodszki12, Paula J Busse13, Anette Bygum14,15, Teresa Caballero16, Mauro Cancian17, Anthony Castaldo11, Danny M Cohn18, Dorottya Csuka19, Henriette Farkas19, Mark Gompels20, Richard Gower21, Anete S Grumach22, Guillermo Guidos-Fogelbach23, Michihiro Hide24,25, Hye-Ryun Kang26, Allen Phillip Kaplan27, Constance Katelaris28, Sorena Kiani-Alikhan29, Wei-Te Lei30, Richard Lockey31, Hilary Longhurst32, William R Lumry33, Andrew MacGinnitie34, Alejandro Malbran35, Inmaculada Martinez Saguer36, Juan José Matta37, Alexander Nast38, Dinh Nguyen39, Sandra A Nieto-Martinez40, Ruby Pawankar41, Jonathan Peter42,43, Grzegorz Porebski44, Nieves Prior45, Avner Reshef46, Marc Riedl47, Bruce Ritchie48, Farrukh Rafique Sheikh49, William B Smith50, Peter J Spaeth51, Marcin Stobiecki44, Elias Toubi52, Lilian Agnes Varga19, Karsten Weller1,2, Andrea Zanichelli53, Yuxiang Zhi54, Bruce Zuraw55, Timothy Craig56.
Abstract
Hereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1 inhibitor (type 1) and HAE with dysfunctional C1 inhibitor (type 2), by providing guidance on common and important clinical issues, such as: (1) How should HAE be diagnosed? (2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? (3) What are the goals of treatment? (4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast-feeding women? and (5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.Entities:
Keywords: C1 inhibitor; DELPHI; disease control; guideline; hereditary angioedema; management
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Year: 2022 PMID： 35006617 DOI： 10.1111/all.15214
Source DB: PubMed Journal: Allergy ISSN： 0105-4538 Impact factor: 14.710