Advertisement

Recurrent Edema

  • Peter JandusEmail author
Chapter

Abstract

The most common triggers of angioedema include mechanical trauma, mental stress, and airway infections

Hereditary angioedema (HAE) is caused by deficiency or dysfunction of the SERPING1 gene and is inherited in an autosomal dominant pattern

Three types of HAE have been described: type 1, characterized by low production of functionally active C1 esterase inhibitor (C1-INH), type 2 characterized by normal or elevated levels of C1-INH but with functional impairment of the protein, and type 3 present with similar clinical manifestations, but no abnormalities in C1-INH level or function

Symptoms often worsen during puberty, introduction of estrogen-containing medications for acne or birth control, in females

Plasma-derived C1-INH, like Berinert and Cinryze can be used for the treatment of acute attacks, for short-term or long-term prophylaxis of HEA by taking into account local approvals

Keywords

Hereditary angioedema C1 esterase inhibitor Berinert 

References

  1. 1.
    Farkas H, Martinez-Saguer I, Bork K, Bowen T, Craig T, Frank M, Germenis AE, Grumach AS, Luczay A, Varga L, Zanichelli A, HAWK. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Allergy. 2017;72(2):300–13.CrossRefGoogle Scholar
  2. 2.
    Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine. 1992;71(4):206–15.CrossRefGoogle Scholar
  3. 3.
    Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267–74.CrossRefGoogle Scholar
  4. 4.
    Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129(11):1420–7.CrossRefGoogle Scholar
  5. 5.
    Speletas M, Szilagyi A, Psarros F, Moldovan D, Magerl M, Kompoti M, Gramoustianou E, Bors A, Mihaly E, Tordai A, Avramouli A, Varga L, Maurer M, Farkas H, Germenis AE. Hereditary angioedema: molecular and clinical differences among European populations. J Allergy Clin Immunol. 2015;135(2):570–3.CrossRefGoogle Scholar
  6. 6.
    Zuraw BL, Herschbach J. Detection of C1 inhibitor mutations in patients with hereditary angioedema. J Allergy Clin Immunol. 2000;105(3):541–6.CrossRefGoogle Scholar
  7. 7.
    Pappalardo E, Cicardi M, Duponchel C, Carugati A, Choquet S, Agostoni A, Tosi M. Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. J Allergy Clin Immunol. 2000;106(6):1147–54.CrossRefGoogle Scholar
  8. 8.
    Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 2008;359(10):1027–36.CrossRefGoogle Scholar
  9. 9.
    Morgan BP. Hereditary angioedema–therapies old and new. N Engl J Med. 2010;363(6):581–3.CrossRefGoogle Scholar
  10. 10.
    Zotter Z, Csuka D, Szabo E, Czaller I, Nebenfuhrer Z, Temesszentandrasi G, Fust G, Varga L, Farkas H. The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency. Orphanet J Rare Dis. 2014;9:44.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of Immunology and Allergology, Department of Internal MedicineUniversity Hospital and Medical FacultyGenevaSwitzerland

Personalised recommendations