title: Rituximab in severe pemphigus.
abstract: Pemphigus is a life-threatening autoimmune bullous disorder that requires long-term treatment with systemic corticosteroids mostly in conjunction with further immunosuppressants. Rituximab has opened a new avenue for severe and refractory pemphigus patients. In 95% of the 136 pemphigus patients reported to date in the English, French, and German literature, at least a partial remission has been achieved. In about two-thirds of communicated cases, application of rituximab resulted in the healing of all lesions. A controlled prospective study, however, has only recently being initiated. In pemphigus, rituximab is aimed at resetting the immune system to a condition in which autoreactive B lymphocytes are eliminated or sufficiently controlled. Here, we review mechanisms of action, different treatment protocols, clinical responses, and the safety profile of rituximab.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. enno.schmidt@uk-sh.de
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Matthias
authors.initials: M
authors.lastname: Goebeler
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
mesh_terms: D000911:Antibodies, Monoclonal / Q000009:adverse effects / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20 / Q000276:immunology*; D001424:Bacterial Infections / Q000139:chemically induced; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use; D010392:Pemphigus / Q000188:drug therapy* / Q000473:pathology; D012074:Remission Induction; D000069283:Rituximab; D018805:Sepsis / Q000139:chemically induced; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007155:Immunologic Factors; D000069283:Rituximab