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Hodl, I; Bosch, P; Dreo, B; Stradner, MH.
Case Report: Extensive Phosphorylation of Interleukin-1 Receptor-Associated Kinase 4 in a Patient With Schnitzler Syndrome.
Front Immunol. 2020; 11: 576200-576200. Doi: 10.3389/fimmu.2020.576200 (- Case Report) [OPEN ACCESS]
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Leading authors Med Uni Graz: Hodl Isabel; Stradner Martin Helmut
Co-authors Med Uni Graz: Bosch Philipp; Dreo Barbara
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Abstract:: Schnitzler syndrome (SchS) is a rare autoinflammatory disease, characterized by urticarial rash, recurrent fever, osteo-articular pain/arthritis with bone condensation, and monoclonal gammopathy. Diagnosis may be difficult due to overlapping signs with other diseases. Here, we describe the case of a 62-year-old man with SchS, who was initially misdiagnosed with multicentric Castleman disease (MCD). As excessive release of IL-6 is characteristic of MCD, in contrast to IL-1 in SchS, we measured the phosphorylation of intracellular signaling proteins of the respective pathways by flow cytometry. We found a distinct increase of phosphorylated IRAK-4 in our patient's B cells and monocytes while phosphorylation of STAT-3 was low, suggesting predominant IL-1 signaling. In accordance with these results and the classification criteria, we established the diagnosis of SchS instead of MCD and commenced therapy with the IL-1 receptor antagonist anakinra. We observed a rapid remission of signs accompanied by a reduction of phosphorylated IRAK-4 to normal levels. In conclusion, we propose phosphorylated IRAK-4 in B cells and monocytes as a potential marker for diagnosis of SchS and for treatment response to IL-1 blockade. Copyright © 2020 Hodl, Bosch, Dreo and Stradner.
Find related publications in this database (Keywords): Schnitzler syndrome; autoinflammatory disease; interleukin-1 receptor associated kinase 4; flow cytometry; interleukin 1; Castleman disease