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Angioedema and emergency medicine: From pathophysiology to diagnosis and treatment

  • Federica Depetri
    Affiliations
    Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milano, Italy
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  • Alberto Tedeschi
    Affiliations
    Unità Operativa di Medicina Generale, Ospedale Bolognini, ASST Bergamo Est, Seriate, Bergamo, Italy
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  • Massimo Cugno
    Correspondence
    Corresponding author at: Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca’ Granda Via Pace, 9, 20122 Milano, Italy.
    Affiliations
    Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milano, Italy
    Search for articles by this author
Published:September 13, 2018DOI:https://doi.org/10.1016/j.ejim.2018.09.004

      Highlights

      • Angioedema is a self-limiting edema of the subcutaneous or submucosal tissues.
      • Angioedema of larynx is life-threatening while angioedema of bowel is very painful.
      • Angioedema can be acquired or hereditary and histamine- or bradykinin-mediated.
      • First line therapy consists of epinephrine, antihistamines and corticosteroids.
      • C1-inhibitor, ecallantide, icatibant or fresh frozen plasma are second line therapies.

      Abstract

      Angioedema is a self-limiting edema of the subcutaneous or submucosal tissues due to localised increase of microvascular permeability whose mediator may be histamine or bradykinin. Patients present to emergency department when angioedema involves oral cavity and larynx (life-threatening conditions) or gut (mimicking an acute abdomen).
      After initial evaluation of consciousness and vital signs to manage breathing and to support circulation if necessary, a simple approach can be applied for a correct diagnosis and treatment. Forms of edema such as anasarca, myxedema, superior vena cava syndrome and acute dermatitis should be ruled out. Then, effort should be done to differentiate histaminergic from non-histaminergic angioedema. Concomitant urticaria and pruritus suggest a histaminergic origin. Exposure to allergens and drugs (mainly ACE inhibitors and non steroidal anti-inflammatory drugs) should be investigated as well as a family history of similar symptoms. Allergic histaminergic angioedema has a rapid course (minutes) whereas non histaminergic angioedema is slower (hours). Since frequently the intervention needs to be immediate, the initial diagnosis is only clinical. However, laboratory tests can be subsequently confirmatory.
      Allergic angioedema is sensitive to standard therapies such as epinephrine, glucocorticoids and antihistamines whereas non histaminergic angioedema is often resistant to these drugs. Therapeutic options for angioedema due C1-inhibitor deficiencies are C1-inhibitor concentrates, icatibant and ecallantide. If these drugs are not available, fresh frozen plasma can be considered. All these medications have been used also in ACE inhibitor-induced angioedema with variable results thus they are not currently recommended whereas experts agree on the discontinuation of the causative drug.

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