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Aktuelle Therapie des Pemphigus

Therapy of pemphigus

  • Leitthema
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Zusammenfassung

Hintergrund

Unter den bullösen Autoimmundermatosen stellt der Pemphigus eine potenziell lebensbedrohliche Erkrankungsgruppe dar. Eine schnell wirksame und effektive Therapie ist daher entscheidend.

Ziel der Arbeit

In dieser Übersichtsarbeit werden die aktuellen Therapien unter Berücksichtigung der Leitlinien erörtert und neue Therapieoptionen vorgestellt.

Methoden

Es erfolgte eine Literaturrecherche.

Ergebnisse

Die Basistherapie des Pemphigus sind systemische Glukokortikosteroide, zudem steht eine Reihe von adjuvanten Immunsuppressiva wie Azathioprin und Mycophenolat-Mofetil/Mycophenolsäure zur Steroideinsparung zur Verfügung. In einer kürzlichen prospektiven randomisierten Studie wurde die Wirkung des Anti-CD20-Antikörpers Rituximab eindrucksvoll gezeigt. In schweren oder refraktären Fällen werden Immunadsorption oder hoch dosierte intravenöse Immunglobuline (IVIG) empfohlen. Eine adjuvante Immunadsorption erscheint auch bei Patienten mit hohen Autoantikörperspiegeln in den ersten 8 bis 12 Wochen der Therapie sinnvoll. Diverse neue Therapeutika befinden sich derzeit in der klinischen Erprobung.

Diskussion

Die Therapie des Pemphigus hat sich durch den Einsatz von Rituximab deutlich verbessert. Hierdurch kann der Einsatz von Glukokortikosteroiden, der mit einer hohen Rate an unerwünschten Nebenwirkungen und erhöhter Mortalität assoziiert war, reduziert werden. Nach einer Zulassung von Rituximab zur Behandlung des Pemphigus durch die amerikanische FDA (Food and Drug Administration) 2018 wird die europäische Zulassung für 2019 erwartet.

Abstract

Background

Pemphigus diseases are a heterogeneous group of potentially life-threatening autoimmune bullous disorders. Therefore, rapidly acting and effective therapeutic approaches are essential.

Objectives

In this review, current therapeutic options in line with available guidelines are presented and new therapeutic approaches are discussed.

Methods

A literature search was performed using PubMed.

Results

Treatment of pemphigus is based on systemic glucocorticosteroids, frequently combined with potentially corticosteroid-sparing immunosuppressants such as azathioprine and mycophenolate mofetil/mycophenolic acid. Recently, the impressive efficacy of the anti-CD20 antibody rituximab has been shown in a prospective randomized trial. In severe or treatment-refractory cases, immunoadsorption or high-dose intravenous immunoglobulins (IVIG) are recommended. Adjuvant immunoadsorption also seems to be useful within the first 8–12 weeks of therapy in patients with very high autoantibody levels. A variety of new therapeutic approaches is currently evaluated in phase IIa studies.

Conclusion

Therapy of pemphigus has been greatly improved by the employment of rituximab. The use of glucocorticosteroids, associated with a high number of adverse events and elevated mortality, could be reduced by the additional use of rituximab. After approval of rituximab for the treatment of pemphigus by the US Food and Drug Administration in 2018, licensing in Europe is expected in 2019.

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Authors and Affiliations

  1. Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    N. van Beek MD, D. Zillikens & E. Schmidt

  2. Lübecker Institut für Experimentelle Dermatologie (LIED), Universität zu Lübeck, Lübeck, Deutschland

    E. Schmidt

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  1. N. van Beek MD
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  2. D. Zillikens
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  3. E. Schmidt
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Correspondence to N. van Beek MD.

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N. van Beek, D. Zillikens und E. Schmidt geben an, dass kein Interessenkonflikt besteht.

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

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van Beek, N., Zillikens, D. & Schmidt, E. Aktuelle Therapie des Pemphigus. Hautarzt 70, 243–253 (2019). https://doi.org/10.1007/s00105-019-4385-9

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