Case report: A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy.

Autor: Wiedenmayer N; Department of Dermatology, University Hospital Heidelberg, Heidelberg, Germany., Hogrefe K; Department of Dermatology, University Hospital Heidelberg, Heidelberg, Germany., Mihalceanu S; Department of Dermatology, University Hospital Heidelberg, Heidelberg, Germany., Winkler JK; Department of Dermatology, University Hospital Heidelberg, Heidelberg, Germany., Enk AH; Department of Dermatology, University Hospital Heidelberg, Heidelberg, Germany.
Jazyk: angličtina
Zdroj: The Journal of dermatology [J Dermatol] 2024 Dec; Vol. 51 (12), pp. 1665-1668. Date of Electronic Publication: 2024 Sep 30.
DOI: 10.1111/1346-8138.17475
Abstrakt: Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid-sparing immunosuppressants, or intravenous monoclonal anti-CD20-antibody therapy. The European guidelines suggest high-dose intravenous immunoglobulin (IVIg) therapy as a promising approach for severe or treatment-resistant cases. We report on a 65-year-old woman with severe and recurrent disease who achieved long-term disease stabilization with IVIg treatment. Because of recurrent fatigue and headache, the patient was switched to an alternative IVIg preparation with a novel manufacturing process, thus ensuring high purity and better tolerability. We observed excellent efficacy, yet side effects remained largely unchanged. Further studies are necessary to evaluate the long-term efficacy and tolerability of this new IVIg preparation.
(© 2024 The Author(s). The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)
Databáze: MEDLINE