Publication

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome

Journal Paper/Review - Feb 10, 2016

Units
PubMed
Doi

Citation
Sönnichsen A, Cozzio A, Hoetzenecker W, Schmid-Grendelmeier P, Petrausch U, Chang Y, Desislava I, Mehra T, Kerl K, Mangana J, Saulite I, Guenova E. Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome. J Dermatolog Treat 2016; 27:436-8.
Type
Journal Paper/Review (English)
Journal
J Dermatolog Treat 2016; 27
Publication Date
Feb 10, 2016
Issn Electronic
1471-1753
Pages
436-8
Brief description/objective

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.