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      Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis

      review-article
      Author(s): I , * , II , I , III , IV , V , VI , II , II , VII , VIII , I , V , II , IX ,   II , I , X , I , IV , XI , XII , I , XIII , VII , X , V
      Publication date (Electronic):
      Journal: Clinics
      Publisher: Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo
      Keywords: Hereditary Angioedema, Angioedema, C1 Inhibitor Deficiency, Coagulation Factor XII Mutations, Management, Guidelines

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          Abstract

          Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Intestinal angioedema is another important and incapacitating presentation that may be the main or only manifestation during an attack. In this article, a group of experts from the “Associação Brasileira de Alergia e Imunologia (ASBAI)” and the “Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH)” has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

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          Most cited references86

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          The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

          T Zuberbier, W. Aberer, R Asero … (2014)
          This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
          Article 0 comments Cited 275 times – based on 0 reviews     Review now
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            Hereditary angioedema: new findings concerning symptoms, affected organs, and course.

            Jochen Hardt, G. Meng, Petra Staubach … (2006)
            Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Our aim was to examine a temporal and spatial pattern of the edema episodes by evaluating the long-term course of hereditary angioedema in order to establish a specific swelling pattern. Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. A total of 131110 edema episodes were observed. Clinical symptoms started at a mean age of 11.2 (SD 7.7) years. During the following cumulative 5736 years, only 370 (6.5%) symptom-free years occurred. Skin swellings, including extremity, facial, genital, and trunk swellings, and abdominal attacks occurred in 97.4% of all edema episodes of the disease. The other episodes were laryngeal edema (0.9%); edema of the soft palate (0.6%); tongue swellings (0.3%); headache episodes (0.7%); episodes affecting urinary bladder (0.3%), chest (0.2%), muscles (0.4%), joints (0.1%), kidneys (0.1%), and esophagus (0.05%), and were partly combined with other edema episodes. The per-patient analysis and the per-episode analysis revealed markedly discrepant results. On average, women had a more severe course of the disease than men. Patients with early onset of clinical symptoms were affected more severely than those with late onset. The described swelling pattern is specific for HAE and allows a tentative diagnosis based on clinical symptoms and the course of the disease.
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              Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema.

              Marco Cicardi, Aleena Banerji, Francisco Bracho … (2010)
              Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days. Icatibant was given once, subcutaneously, at a dose of 30 mg. The primary end point was the median time to clinically significant relief of symptoms. A total of 56 and 74 patients underwent randomization in the FAST-1 and FAST-2 trials, respectively. The primary end point was reached in 2.5 hours with icatibant versus 4.6 hours with placebo in the FAST-1 trial (P=0.14) and in 2.0 hours with icatibant versus 12.0 hours with tranexamic acid in the FAST-2 trial (P<0.001). In the FAST-1 study, 3 recipients of icatibant and 13 recipients of placebo needed treatment with rescue medication. The median time to first improvement of symptoms, as assessed by patients and by investigators, was significantly shorter with icatibant in both trials. No icatibant-related serious adverse events were reported. In patients with hereditary angioedema having acute attacks, we found a significant benefit of icatibant as compared with tranexamic acid in one trial and a nonsignificant benefit of icatibant as compared with placebo in the other trial with regard to the primary end point. The early use of rescue medication may have obscured the benefit of icatibant in the placebo trial. (Funded by Jerini; ClinicalTrials.gov numbers, NCT00097695 and NCT00500656.)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Clinics (Sao Paulo)
                Journal ID (iso-abbrev): Clinics (Sao Paulo)
                Title: Clinics
                Publisher: Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo
                ISSN (Print): 1807-5932
                ISSN (Electronic): 1980-5322
                Publication date (Electronic): 21 April 2018
                Publication date (Print): 2018
                Volume: 73
                Electronic Location Identifier: e310
                Affiliations
                [I ]Divisao de Imunologia Clinica e Alergia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR
                [II ]Faculdade de Medicina de Ribeirao Preto, Universidade de Sao Paulo, Ribeirao Preto, SP, BR
                [III ]Departamento de Medicina Interna e Suporte Diagnostico, Faculdade de Medicina da Bahia, Salvador, BA, BR
                [IV ]Departamento de Pediatria, Universidade Federal do Parana, Curitiba, PR, BR
                [V ]Imunologia Clinica, Faculdade de Medicina do ABC, Santo Andre, SP, BR
                [VI ]Hospital do Servidor Publico Estadual Francisco Morato Oliveira, Sao Paulo, SP, BR
                [VII ]Divisao de Imunologia, Departamento de Medicina Interna, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, BR
                [VIII ]Departamento de Pediatria, Divisao de Imunologia Clinica e Alergia – Universidade Federal de Minas Gerais (UFMG), Minas Gerais, MG, BR
                [IX ]Divisao de Imunologia Clinica e Alergia, Departamento de Medicina Interna, Faculdade de Ciencias Medicas, Universidade Estadual de Campinas, Campinas, SP, BR
                [X ]Departamento de Biofisica, Universidade Federal de Sao Paulo, Sao Paulo, SP, BR
                [XI ]Escola Superior de Ciencias da Santa Casa de Misericordia de Vitoria, Vitoria, ES, BR
                [XII ]Divisao de Alergia, Imunologia e Reumatologia Clinica, Departamento de Pediatria, Universidade Federal de Sao Paulo, Sao Paulo, SP, BR
                [XIII ]Divisao de Alergia e Imunologia Clinica, Faculdade de Medicina de Sao Jose do Rio Preto, Sao Jose do Rio Preto, SP, BR
                Author notes
                *Corresponding author. E-mail: pbianchi@ 123456usp.br
                Article
                Publisher ID: cln_73p1
                DOI: 10.6061/clinics/2018/e310
                PMC ID: 5910635
                PubMed ID: 29723342
                SO-VID: b246b62a-4fb2-4487-9629-f75e009cf26d
                Copyright © Copyright © 2018 CLINICS
                License:

                This is an Open Access article distributed under the terms of the Creative Commons License ( http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.

                History
                Date received : 2 August 2017
                Date accepted : 18 December 2017
                Categories
                Subject: Review Article

                ScienceOpen disciplines: Medicine
                Keywords: hereditary angioedema,angioedema,c1 inhibitor deficiency,coagulation factor xii mutations,management, guidelines
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: hereditary angioedema, angioedema, c1 inhibitor deficiency, coagulation factor xii mutations, management, guidelines

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