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Der Hautarzt

, Volume 70, Issue 2, pp 107–115 | Cite as

Prophylaxe von Angioödemen

  • V. Zampeli
  • M. MagerlEmail author
Leitthema
  • 78 Downloads

Zusammenfassung

Das Angioödem ist eine spontan auftretende, ödematöse Schwellung der tieferen Schichten der Haut oder der Schleimhaut. Angioödeme im Bereich der Atemwege sind potenziell lebensbedrohlich. Die Zuordnung von Angioödemen in Mastzell-vermittelt (z. B. Urtikaria) oder Bradykinin-vermittelt (z. B. hereditäres Angioödem) ist wichtig für die richtige und rationale Behandlung. Grundsätzlich stehen dafür 2 therapeutische Strategien zur Verfügung: Zum einen die bedarfsweise Behandlung von bereits aufgetretenen Angioödemen mit dem Ziel, die Weiterentwicklung der Attacke zu beenden und damit die Schwere und Dauer der Attacke zu begrenzen. Diese Strategie ist in der Versorgung von Patienten mit hereditärem Angioödem fest verankert, bei der chronischen spontanen Urtikaria hingegen findet die Bedarfstherapie keine Anwendung. Demgegenüber hat die Strategie der Prophylaxe zum Ziel, das Auftreten von spontanen und induzierten Attacken möglichst zu verhindern. Die Prophylaxe ist alleinige Therapiestrategie bei der chronischen Urtikaria und zieht sich durch alle Stufen des Behandlungsalgorithmus. Beim hereditären Angioödem kann die Bedarfstherapie nach sorgfältiger und individueller Indikationsstellung durch eine Prophylaxe komplementiert werden. In dieser Indikation gewinnt sie aktuell durch verbesserte Behandlungsoptionen zunehmend an Bedeutung. Patienten, die ein prophylaktisches Regime anwenden, sind viel seltener gezwungen, auf das unvorhersehbare Auftreten einer Attacke zu warten und dann zu reagieren. Die prophylaktische Behandlung findet – im Gegensatz zur bedarfsweisen Therapie – zu von dem Patienten selbst bestimmten Zeiten statt. Der Wegfall der Unvorhersehbarkeit ist ein entscheidendes Moment der Lebensqualitätsverbesserung.

Schlüsselwörter

C1-Inhibitor-Mangel Chronische spontane Urtikaria Bedarfstherapie Kurzzeitprophylaxe Langzeitprophylaxe 

Angioedema prophylaxis

Abstract

Angioedema is a spontaneous, edematous swelling of the deep layers of the skin or mucous membrane. Angioedema in the respiratory tract is potentially life-threatening. The classification of angioedema into mast-cell-mediated (e. g. urticaria) or bradykinin-mediated (e. g. hereditary angioedema) is important for correct and rational treatment. Generally, two therapeutic strategies are available for angioedema treatment. On-demand treatment of angioedema symptoms that already have emerged aims to stop the further development of the attack and, thus, limits the severity and duration of the attack. This strategy is well established in the treatment of patients with hereditary angioedema, whereas in chronic spontaneous urticaria on-demand therapy plays no role in the guideline recommendations. In contrast, the therapeutic strategy of prophylaxis aims to prevent the occurrence of spontaneous and induced attacks as far as possible. Prophylaxis is the sole therapy strategy for chronic urticaria and is applied at all stages of the treatment algorithm. In the case of hereditary angioedema, on-demand therapy can be complemented by prophylaxis after careful and individual indication. In hereditary angioedema, prophylaxis is currently gaining in importance due to improved treatment options. Patients who use a prophylactic regime are much less likely to be forced to wait for the unpredictable occurrence of an attack and then to react with an on-demand treatment. Prophylactic treatment takes place at times determined by the patient himself, in contrast to treatment on an as-needed basis. The loss of unpredictability is a decisive moment in improving the quality of life.

Keywords

C1-Inhibitor deficiency Chronic spontaneous urticaria On-demand therapy Short-term prophylaxis Long-term prophylaxis 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

V. Zampeli hat persönliche Honorare von Shire, CSL Behring und Pharming erhalten. M. Magerl hat persönliche Honorare und nichtfinanzielle Unterstützung von BioCryst, CSL Behring, KalVista, Pharming und Shire erhalten.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

Literatur

  1. 1.
    Akenroye AT, Mcewan C, Saini SS (2018) Montelukast reduces symptom severity and frequency in patients with angioedema-predominant chronic spontaneous urticaria. J Allergy Clin Immunol Pract 6:1403–1405CrossRefGoogle Scholar
  2. 2.
    Aygoren-Pursun E, Martinez Saguer I, Kreuz W et al (2013) Risk of angioedema following invasive or surgical procedures in HAE type I and II—the natural history. Allergy 68:1034–1039CrossRefGoogle Scholar
  3. 3.
    Azofra J, Diaz C, Antepara I et al (2015) Positive response to omalizumab in patients with acquired idiopathic nonhistaminergic angioedema. Ann Allergy Asthma Immunol 114:418–419e1CrossRefGoogle Scholar
  4. 4.
    Bork K, Aygören-Pürsün E, Bas M et al (2019) Leitlinie „Hereditäres Angioödem durch C1-Inhibitor-Mangel“. Allergo J Int 28: (in Druck)Google Scholar
  5. 5.
    Bork K, Bygum A, Hardt J (2008) Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients. Ann Allergy Asthma Immunol 100:153–161CrossRefGoogle Scholar
  6. 6.
    Bork K, Hardt J, Staubach-Renz P et al (2011) Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 112:58–64CrossRefGoogle Scholar
  7. 7.
    Bork K, Pitton M, Harten P et al (1999) Hepatocellular adenomas in patients taking danazol for hereditary angio-oedema. Lancet 353:1066–1067CrossRefGoogle Scholar
  8. 8.
    Bork K, Witzke G (1989) Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency. J Allergy Clin Immunol 83:677–682CrossRefGoogle Scholar
  9. 9.
    Bork K, Wulff K, Hardt J et al (2009) Hereditary angioedema caused by missense mutations in the factor XII gene: clinical features, trigger factors, and therapy. J Allergy Clin Immunol 124:129–134CrossRefGoogle Scholar
  10. 10.
    Bork K, Wulff K, Witzke G et al (2017) Treatment for hereditary angioedema with normal C1-INH and specific mutations in the F12 gene (HAE-FXII). Allergy 72:320–324CrossRefGoogle Scholar
  11. 11.
    Brunetta E, Shiffer D, Folci M et al (2018) Omalizumab for idiopathic nonhistaminergic angioedema: evidence for efficacy in 2 patients. Case Reports Immunol 2018:8067610CrossRefGoogle Scholar
  12. 12.
    Bygum A, Vestergaard H (2013) Acquired angioedema—occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. Int Arch Allergy Immunol 162:149–155CrossRefGoogle Scholar
  13. 13.
    Deroux A, Boccon-Gibod I, Fain O et al (2016) Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema. Clin Exp Immunol 185:332–337CrossRefGoogle Scholar
  14. 14.
    Dreyfus DH, Na CR, Randolph CC et al (2014) Successful rituximab B lymphocyte depletion therapy for angioedema due to acquired C1 inhibitor protein deficiency: association with reduced C1 inhibitor protein autoantibody titers. Isr Med Assoc J 16:315–316PubMedGoogle Scholar
  15. 15.
    Frank MM, Sergent JS, Kane MA et al (1972) Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study. N Engl J Med 286:808–812CrossRefGoogle Scholar
  16. 16.
    Gericke J, Metz M, Ohanyan T et al (2017) Serum autoreactivity predicts time to response to omalizumab therapy in chronic spontaneous urticaria. J Allergy Clin Immunol 139:1059–1061.e1CrossRefGoogle Scholar
  17. 17.
    Greve J, Strassen U, Gorczyza M et al (2016) Prophylaxis in hereditary angioedema (HAE) with C1 inhibitor deficiency. J Dtsch Dermatol Ges 14:266–275PubMedGoogle Scholar
  18. 18.
    Groner A, Nowak T, Schafer W (2012) Pathogen safety of human C1 esterase inhibitor concentrate. Transfusion 52:2104–2112CrossRefGoogle Scholar
  19. 19.
    Hosea SW, Santaella ML, Brown EJ et al (1980) Long-term therapy of hereditary angioedema with danazol. Ann Intern Med 93:809–812CrossRefGoogle Scholar
  20. 20.
    Longhurst H, Cicardi M, Craig T et al (2017) Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor. N Engl J Med 376:1131–1140CrossRefGoogle Scholar
  21. 21.
    Longhurst HJ, Farkas H, Craig T et al (2010) HAE international home therapy consensus document. Allergy Asthma Clin Immunol 6:22CrossRefGoogle Scholar
  22. 22.
    Magerl M, Germenis AE, Maas C et al (2017) Hereditary angioedema with normal C1 inhibitor: update on evaluation and treatment. Immunol Allergy Clin North Am 37:571–584CrossRefGoogle Scholar
  23. 23.
    Maurer M, Magerl M (2011) Long-term prophylaxis of hereditary angioedema with androgen derivates: a critical appraisal and potential alternatives. J Dtsch Dermatol Ges 9:99–107PubMedGoogle Scholar
  24. 24.
    Maurer M, Magerl M, Ansotegui I et al (2018) The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update. Allergy 73:1575–1596CrossRefGoogle Scholar
  25. 25.
    Maurer M, Metz M, Magerl M et al (2004) Autoreactive urticaria and autoimmune urticaria. Hautarzt 55:350–356CrossRefGoogle Scholar
  26. 26.
    Muhlberg H, Ettl N, Magerl M (2016) An analysis of the teaching of intravenous self-administration in patients with hereditary angio-oedema. Clin Exp Dermatol 41:366–371CrossRefGoogle Scholar
  27. 27.
    Spaulding WB (1960) MEthyltestosterone therapy for hereditary episodic edema (hereditary angioneurotic edema). Ann Intern Med 53:739–745CrossRefGoogle Scholar
  28. 28.
    Symons C, Rossi O, Magerl M et al (2013) Practical approach to self-administration of intravenous C1-INH concentrate: a nursing perspective. Int Arch Allergy Immunol 161(Suppl 1):17–20CrossRefGoogle Scholar
  29. 29.
    Varga L, Fust G, Csuka D et al (2011) Treatment with C1-inhibitor concentrate does not induce IgM type anti-C1 inhibitor antibodies in patients with hereditary angioedema. Mol Immunol 48:572–576CrossRefGoogle Scholar
  30. 30.
    Vitrat-Hincky V, Gompel A, Dumestre-Perard C et al (2010) Type III hereditary angio-oedema: clinical and biological features in a French cohort. Allergy 65:1331–1336CrossRefGoogle Scholar
  31. 31.
    Weller K, Magerl M, Maurer M (2011) Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. J Eur Acad Dermatol Venereol 25:119–120CrossRefGoogle Scholar
  32. 32.
    Wong BN, Vadas P (2017) Angioedema suppressed by a combination of anti-histamine and leukotriene modifier. Allergy Asthma Clin Immunol 13:28CrossRefGoogle Scholar
  33. 33.
    Zanichelli A, Azin GM, Wu MA et al (2017) Diagnosis, course, and management of angioedema in patients with acquired C1-inhibitor deficiency. J Allergy Clin Immunol Pract 5:1307–1313CrossRefGoogle Scholar
  34. 34.
    Zuberbier T, Aberer W, Asero R et al (2018) The EAACI/GA(2)LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria. Allergy 73:1393–1414CrossRefGoogle Scholar
  35. 35.
    Zuraw BL, Busse PJ, White M et al (2010) Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. N Engl J Med 363:513–522CrossRefGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2019

Authors and Affiliations

  1. 1.Klinik für Dermatologie, Venerologie und Allergologie, Allergie-Centrum-Charité/ECARFCharité – Universitätsmedizin BerlinBerlinDeutschland

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