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Therapieoptimierung bei schweren bullösen Autoimmundermatosen

Optimizing therapy in patients with severe autoimmune blistering skin diseases

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Zusammenfassung

Bullöse Autoimmundermatosen sind eine heterogene Gruppe von Erkrankungen. Nach der Höhe der Spaltbildung wird der mit intraepidermalen Blasen einhergehende Pemphigus von den subepidermal blasenbildenden Erkrankungen abgegrenzt, die sich in Pemphigoiderkrankungen, Epidermolysis bullosa acquisita (EBA) und Dermatitis herpetiformis gliedern. Während sich die Krankheitsaktivität bei den meisten dieser Erkrankungen durch topische oder systemische Kortikosteroide und zusätzliche Gabe von Immunsuppressiva/-modulatoren wie Dapson, Doxyzyklin, Azathioprin, Mycophenolatmofetil oder Methotrexat in der Regel ausreichend unterdrücken lässt, stellen Pemphigus, Schleimhautpemphigoid und EBA eine therapeutische Herausforderung dar. Nur bei einer Minderheit dieser Patienten reicht eine konventionelle immunsuppressive Therapie aus, um eine klinische Remission zu erzielen. Als potente Second-line-Therapie standen bis vor wenigen Jahren nur Cyclophosphamid und hoch dosierte intravenöse Immunglobuline (IVIG) zur Verfügung. Immunadsorption und der monoklonale anti-CD20-Antikörper Rituximab haben sich als weitere Therapieoptionen etabliert. Die vorliegende Übersicht fasst die aktuellen Erkenntnisse zu Effektivität, Nebenwirkungen, Behandlungsprotokollen und Wirkmechanismen von IVIG, Immunadsorption und Rituximab als potente Therapieoptionen schwerer und/oder therapierefraktärer bullöser Autoimmundermatosen zusammen.

Abstract

Autoimmune bullous diseases are a heterogeneous group of disorders that can be subdivided according to the level of split formation in the intraepidermal blistering pemphigus diseases and subepidermal bullous disorders, latter including pemphigoid diseases, epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis. In the majority of autoimmune bullous disorders, disease activity can be sufficiently controlled by systemic corticosteroids in combination with further immunsuppressants/-modulants such as dapsone, doxycycline, azathioprine, mycophenolate mofetil, or methotrexate. In contrast, in pemphigus, mucous membrane pemphigoid, and EBA, treatment is challenging and conventional immunosuppressive therapy induces clinical remission only in a minority of patients. Until recently, only cyclosphosphamide and high-dose intravenous immunoglobulin (IVIG) were available as potent second-line therapies. Meanwhile, immunoadsorption and the monoclonal anti-CD20 antibody rituximab have been established as further therapeutic options. The present review focuses on efficacy, adverse events, treatment protocols, and mechanisms of action of IVIG, immunoadsorption, and rituximab in the treatment of severe and/or refractory patients with bullous autoimmune diseases.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Der Autor hat an einem von Roche gesponserten Konsensustreffen (2006) teilgenommen. Der Autor hat Honorar (weniger als 4000 EUR) aus Publikationsbeihilfen von Roche bekommen.

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  1. Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    E. Schmidt

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Schmidt, E. Therapieoptimierung bei schweren bullösen Autoimmundermatosen. Hautarzt 60, 633–640 (2009). https://doi.org/10.1007/s00105-008-1680-2

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