Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative

Background: Hereditary angioedema (HAE), a potentially life-threatening genetic disorder due to C1 inhibitor deficiency in most cases, is characterized by sudden and/or recurrent attacks of angioedema (subcutaneous/submucosal swellings). The global World Allergy Organization (WAO)/European Academy o...

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Main Authors: Marcus Maurer, MD, Mohamed Abuzakouk, MD, PhD, Mona Al-Ahmad, MD, Waleed Al-Herz, MD, Hassan Alrayes, MD, Salem Al-Tamemi, MD, Rand Arnaout, MD, Hend Binghadeer, MD, Ravi Gutta, MD, Carla Irani, MD, Hassan Mobayed, MD, Iman Nasr, MD, Hiba Shendi, MD, Fares Zaitoun, MD, FACAAI
Format: Article
Language:English
Published: Elsevier 2023-01-01
Series:World Allergy Organization Journal
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1939455122001053
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author Marcus Maurer, MD
Mohamed Abuzakouk, MD, PhD
Mona Al-Ahmad, MD
Waleed Al-Herz, MD
Hassan Alrayes, MD
Salem Al-Tamemi, MD
Rand Arnaout, MD
Hend Binghadeer, MD
Ravi Gutta, MD
Carla Irani, MD
Hassan Mobayed, MD
Iman Nasr, MD
Hiba Shendi, MD
Fares Zaitoun, MD, FACAAI
author_facet Marcus Maurer, MD
Mohamed Abuzakouk, MD, PhD
Mona Al-Ahmad, MD
Waleed Al-Herz, MD
Hassan Alrayes, MD
Salem Al-Tamemi, MD
Rand Arnaout, MD
Hend Binghadeer, MD
Ravi Gutta, MD
Carla Irani, MD
Hassan Mobayed, MD
Iman Nasr, MD
Hiba Shendi, MD
Fares Zaitoun, MD, FACAAI
author_sort Marcus Maurer, MD
collection DOAJ
description Background: Hereditary angioedema (HAE), a potentially life-threatening genetic disorder due to C1 inhibitor deficiency in most cases, is characterized by sudden and/or recurrent attacks of angioedema (subcutaneous/submucosal swellings). The global World Allergy Organization (WAO)/European Academy of Allergy and Clinical Immunology (EAACI) International guideline for HAE management is comprehensive, but the implementation of this guideline may require regional adaptation considering the diversity in disease awareness, type of medical care systems, and access to diagnostics and treatment. The aim of this Delphi initiative was to build on the global guideline and provide regional adaptation to address the concerns and specific needs in the Middle East. Methods: The Consensus panel comprised 13 experts from the Middle East (3 from the United Arab Emirates, 3 from Saudi Arabia, 2 from Lebanon, 2 from Kuwait, 2 from Oman and 1 from Qatar) who have more than 2 decades of experience in allergy and immunology and are actively involved in managing HAE patients. The process that was carried out to reach the consensus recommendation included: 1.) A systematic literature review for articles related to HAE management using Ovid MEDLINE. 2.) The development of a questionnaire by an internationally acclaimed expert, with 10 questions specific to HAE management in the Middle East. 3.) Experts received the questionnaire via email individually and their answers were recorded (email/interview). 4.) A virtual consensus meeting was organized to discuss the questionnaire, make amends if needed, vote, and achieve consensus. Results: The questionnaire comprised 10 questions, each with 2 or more statements/recommendations on which the regional experts voted. A consensus was reached based on a 70% agreement between the participants. The key highlights include: 1) HAE experts in the Middle East emphasized the importance of a positive family history for arriving at a diagnosis of HAE. 2) The number of episodes per month or per 6-month period and severity should be used, together with other markers, to determine the need for prophylaxis. 3) Disease status should be monitored by periodic visits and the use of patient-reported outcome measures such as the angioedema activity score and the angioedema control test. 4) Attenuated androgens and tranexamic acid may be considered for long-term prophylaxis, if lanadelumab, C1-Inhibitor or berotralstat are not available. Conclusion: This consensus recommendation may help to educate healthcare practitioners in the Middle East and unify their approach to the diagnosis and management of HAE.
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spelling doaj.art-efa1f2180e4a494e808a377acc3a70732023-02-02T04:48:13ZengElsevierWorld Allergy Organization Journal1939-45512023-01-01161100729Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiativeMarcus Maurer, MD0Mohamed Abuzakouk, MD, PhD1Mona Al-Ahmad, MD2Waleed Al-Herz, MD3Hassan Alrayes, MD4Salem Al-Tamemi, MD5Rand Arnaout, MD6Hend Binghadeer, MD7Ravi Gutta, MD8Carla Irani, MD9Hassan Mobayed, MD10Iman Nasr, MD11Hiba Shendi, MD12Fares Zaitoun, MD, FACAAI13Institute of Allergology, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany; Corresponding author. Prof. Marcus Maurer Charité – Universitätsmedizin Berlin | Institute of AllergologyCleveland Clinic, Abu Dhabi- United Arab EmiratesAl-Rashed Allergy Center, Ministry of Health, Kuwait; Microbiology department, College of Medicine, Kuwait University, KuwaitDepartment of Pediatrics, Faculty of Medicine, Kuwait University, KuwaitKing Faisal Specialist Hospital and Research Center, Riyadh, Saudi ArabiaSultan Qaboos University Hospital, Muscat, OmanKing Faisal Specialist Hospital and Research Center, Riyadh, Saudi ArabiaKing Abdulaziz Medical City-Riyadh, Ministry of National Guard Health Affairs, Saudi ArabiaAdult and Pediatric Allergy and Immunology Department, Mediclinic City Hospital, DHCC, DubaiHotel Dieu de France Hospital, St Joseph University, Beirut, LebanonDepartment of Medicine, Hamad Medical Corporation, Doha, QatarRoyal Hospital, Muscat, OmanTawam Hospital, Abu Dhabi, United Arab EmiratesClemenceau Medical Center, Dubai, United Arab EmiratesBackground: Hereditary angioedema (HAE), a potentially life-threatening genetic disorder due to C1 inhibitor deficiency in most cases, is characterized by sudden and/or recurrent attacks of angioedema (subcutaneous/submucosal swellings). The global World Allergy Organization (WAO)/European Academy of Allergy and Clinical Immunology (EAACI) International guideline for HAE management is comprehensive, but the implementation of this guideline may require regional adaptation considering the diversity in disease awareness, type of medical care systems, and access to diagnostics and treatment. The aim of this Delphi initiative was to build on the global guideline and provide regional adaptation to address the concerns and specific needs in the Middle East. Methods: The Consensus panel comprised 13 experts from the Middle East (3 from the United Arab Emirates, 3 from Saudi Arabia, 2 from Lebanon, 2 from Kuwait, 2 from Oman and 1 from Qatar) who have more than 2 decades of experience in allergy and immunology and are actively involved in managing HAE patients. The process that was carried out to reach the consensus recommendation included: 1.) A systematic literature review for articles related to HAE management using Ovid MEDLINE. 2.) The development of a questionnaire by an internationally acclaimed expert, with 10 questions specific to HAE management in the Middle East. 3.) Experts received the questionnaire via email individually and their answers were recorded (email/interview). 4.) A virtual consensus meeting was organized to discuss the questionnaire, make amends if needed, vote, and achieve consensus. Results: The questionnaire comprised 10 questions, each with 2 or more statements/recommendations on which the regional experts voted. A consensus was reached based on a 70% agreement between the participants. The key highlights include: 1) HAE experts in the Middle East emphasized the importance of a positive family history for arriving at a diagnosis of HAE. 2) The number of episodes per month or per 6-month period and severity should be used, together with other markers, to determine the need for prophylaxis. 3) Disease status should be monitored by periodic visits and the use of patient-reported outcome measures such as the angioedema activity score and the angioedema control test. 4) Attenuated androgens and tranexamic acid may be considered for long-term prophylaxis, if lanadelumab, C1-Inhibitor or berotralstat are not available. Conclusion: This consensus recommendation may help to educate healthcare practitioners in the Middle East and unify their approach to the diagnosis and management of HAE.http://www.sciencedirect.com/science/article/pii/S1939455122001053Hereditary angioedemaMiddle eastDelphi consensus
spellingShingle Marcus Maurer, MD
Mohamed Abuzakouk, MD, PhD
Mona Al-Ahmad, MD
Waleed Al-Herz, MD
Hassan Alrayes, MD
Salem Al-Tamemi, MD
Rand Arnaout, MD
Hend Binghadeer, MD
Ravi Gutta, MD
Carla Irani, MD
Hassan Mobayed, MD
Iman Nasr, MD
Hiba Shendi, MD
Fares Zaitoun, MD, FACAAI
Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
World Allergy Organization Journal
Hereditary angioedema
Middle east
Delphi consensus
title Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
title_full Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
title_fullStr Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
title_full_unstemmed Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
title_short Consensus on diagnosis and management of Hereditary Angioedema in the Middle East: A Delphi initiative
title_sort consensus on diagnosis and management of hereditary angioedema in the middle east a delphi initiative
topic Hereditary angioedema
Middle east
Delphi consensus
url http://www.sciencedirect.com/science/article/pii/S1939455122001053
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