title: Schnitzler syndrome: treatment failure to rituximab but response to anakinra.
abstract: Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge. Here, we report a patient who was only recently given the diagnosis of Schnitzler syndrome although the typical symptoms had been present for about 6 years. Administration of the B-cell antibody rituximab did not exert any beneficial effect despite effective elimination of B cells and a reduction of the paraprotein. In contrast, injection of the interleukin-1-receptor antagonist anakinra caused a complete remission within a few days.
authors.affiliation: Department of Dermatology, University of Kiel, Germany.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Eiling
authors.forename: Maike
authors.initials: M
authors.lastname: Möller
authors.forename: Inga
authors.initials: I
authors.lastname: Kreiselmaier
authors.forename: Jochen
authors.initials: J
authors.lastname: Brasch
authors.forename: Thomas
authors.initials: T
authors.lastname: Schwarz
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007279:Injections, Subcutaneous; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors*; D019233:Retreatment; D000069283:Rituximab; D019873:Schnitzler Syndrome / Q000188:drug therapy* / Q000473:pathology; D013997:Time Factors; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007155:Immunologic Factors; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; D000069283:Rituximab; D011239:Prednisolone