The international WAO/EAACI guideline for the management of hereditary angioedemaThe 2017 revision and update

被引：280

作者：

Maurer, M. ^{[1
]}

Magerl, M. ^{[1
]}

Ansotegui, I. ^{[2
]}

Aygoeren-Puersuen, E. ^{[3
]}

Betschel, S. ^{[4
]}

Bork, K. ^{[5
]}

Bowen, T. ^{[6
]}

Boysen, H. Balle ^{[7
]}

Farkas, H. ^{[8
]}

Grumach, A. S. ^{[9
]}

Hide, M. ^{[10
]}

Katelaris, C. ^{[11
,12
]}

Lockey, R. ^{[13
]}

Longhurst, H. ^{[14
]}

Lumry, W. R. ^{[15
]}

Martinez-Saguer, I. ^{[16
]}

Moldovan, D. ^{[17
]}

Nast, A. ^{[18
,19
,20
,21
]}

Pawankar, R. ^{[22
]}

Potter, P. ^{[23
]}

Riedl, M. ^{[24
]}

Ritchie, B. ^{[25
]}

Rosenwasser, L. ^{[26
]}

Sanchez-Borges, M. ^{[27
]}

Zhi, Y. ^{[28
,29
]}

Zuraw, B. ^{[24
,30
]}

Craig, T. ^{[31
]}

机构：

[1] Charite Univ Med Berlin, Dept Dermatol & Allergy, Berlin, Germany

[2] Hosp Quironsalud Bizkaia, Dept Allergy & Immunol, Bilbao, Spain

[3] Univ Hosp Frankfurt, Ctr Children & Adolescents, Frankfurt, Germany

[4] Univ Toronto, St Michaels Hosp, Div Clin Immunol & Allergy, Toronto, ON, Canada

[5] Johannes Gutenberg Univ Mainz, Dept Dermatol, Mainz, Germany

[6] Univ Calgary, Dept Med & Pediat, Calgary, AB, Canada

[7] HAEi, Lausanne, Switzerland

[8] Semmelweis Univ, Hungarian Angioedema Ctr, Dept Internal Med 3, Budapest, Hungary

[9] Fac Med ABC, Clin Immunol, Sao Paulo, Brazil

[10] Hiroshima Univ, Dept Dermatol, Hiroshima, Japan

[11] Campbelltown Hosp, Dept Med, Sydney, NSW, Australia

[12] Western Sydney Univ, Sydney, NSW, Australia

[13] Univ S Florida, Dept Internal Med, Morsani Coll Med, Tampa, FL USA

[14] Cambridge Univ Hosp NHS Fdn Trust, Addenbrookes Hosp, Dept Clin Biochem & Immunol, Cambridge, England

[15] Univ Texas Dallas, Southwestern Med Sch, Allergy Immunol Div, Dept Internal Med, Dallas, TX USA

[16] Hemophilia Ctr Rhine Main, Morfelden Walldorf, Germany

[17] Univ Med & Pharm, Targu Mures, Romania

[18] Berlin Inst Hlth, Dept Dermatol Venereol & Allergy, Div Evidence Based Med dEBM, Berlin, Germany

[19] Free Univ Berlin, Berlin, Germany

[20] Humboldt Univ, Berlin, Germany

[21] Charite Univ Med Berlin, Berlin, Germany

[22] Nippon Med Sch, Dept Pediat, Tokyo, Japan

[23] Univ Cape Town, Dept Med, Cape Town, South Africa

[24] Univ Calif San Diego, Dept Med, La Jolla, CA 92093 USA

[25] Univ Alberta, Div Hematol, Edmonton, AB, Canada

[26] Univ Missouri, Sch Med, Allergy & Immunol Dept, Kansas City, MO 64108 USA

[27] Ctr Med Docente La Trinidad, Allergy & Clin Immunol Dept, Caracas, Venezuela

[28] Peking Union Med Coll Hosp, Dept Allergy, Beijing, Peoples R China

[29] Chinese Acad Med Sci, Beijing, Peoples R China

[30] San Diego VA Healthcare, San Diego, CA USA

[31] Penn State Univ, Dept Med & Pediat, Hershey, PA USA

来源：

ALLERGY | 2018年 / 73卷 / 08期

关键词：

C1-inhibitor; guideline; hereditary angioedema; management; recommendations; C1 ESTERASE INHIBITOR; SHORT-TERM PROPHYLAXIS; ACUTE ATTACKS; C1-INHIBITOR CONCENTRATE; ANGIO-EDEMA; C1-ESTERASE INHIBITOR; ANGIONEUROTIC-EDEMA; ECALLANTIDE TREATMENT; TRANEXAMIC ACID; DOUBLE-BLIND;

D O I：

10.1111/all.13384

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE-1/2 be defined and classified?, (2) How should HAE-1/2 be diagnosed?, (3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, (4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures?

引用

页码：1575 / 1596

页数：22

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