title: Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome.
abstract: Schnitzler syndrome is a rare autoinflammatory disease, which is defined by the presence of two major criteria: chronic urticaria and monoclonal immunoglobulin M (IgM) or immunoglobulin G gammopathy, in combination with at least two additional minor criteria: recurrent fever, leukocytosis and/or elevated C-reactive protein (CRP), objective signs of abnormal bone remodelling and a neutrophilic infiltrate in skin biopsy. We report on a 68-year-old female patient with a 10-year medical history of chronic urticaria, recurrent fever, severe arthralgia and increased CRP. Over the years, multiple diagnostic investigations were performed without conclusive findings, and therapeutic attempts with anti-histamines and several immunosuppressive agents had failed. The decision to initiate monotherapy with interleukin-1 (IL-1) receptor antagonist was based on immunohistochemical detection of the abundance of IL-1β positive cells in the patient's skin biopsy. After starting treatment with anakinra, disappearance of symptoms could be observed within 24 h. Discontinuation of the treatment resulted in a rapid relapse of the symptoms. Finally, already after the initiation of therapy with anakinra, the suspected diagnosis of Schnitzler syndrome could be confirmed by detection of IgM-gammopathy that was initially absent.
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Astrid
authors: A
authors: Sönnichsen
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Ieva
authors: I
authors: Saulite
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Johanna
authors: J
authors: Mangana
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Katrin
authors: K
authors: Kerl
authors: b Medical Directorate, University Hospital of Zürich , Zürich , Switzerland , and.
authors: Tarun
authors: T
authors: Mehra
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Ignatova
authors: I
authors: Desislava
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Yun-Tsan
authors: YT
authors: Chang
authors: c Clinic of Oncology, University Hospital of Zürich , Zürich , Switzerland.
authors: Ulf
authors: U
authors: Petrausch
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Peter
authors: P
authors: Schmid-Grendelmeier
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Wolfram
authors: W
authors: Hoetzenecker
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Antonio
authors: A
authors: Cozzio
authors: a Department of Dermatology , University Hospital of Zürich, University of Zürich , Zürich , Switzerland .
authors: Emmanuella
authors: E
authors: Guenova
mesh_terms: D000368:Aged; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007249:Inflammation / Q000188:drug therapy; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors; D019873:Schnitzler Syndrome / Q000188:drug therapy*
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1
keywords: Schnitzler syndrome; anakinra; interleukin-1 receptor antagonist