[Therapy of pemphigus].

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.