Abstract
IL-1 receptor antagonist anakinra is usually highly efficient in Schnitzler syndrome (SS), a rare inflammatory condition associating urticaria, fever, and IgM monoclonal gammopathy. In this study, we aimed to assess lipopolysaccharide (LPS)-induced production of inflammatory cytokines by peripheral blood mononuclear cells (PBMCs) before and after 1 month of anakinra in patients with SS. LPS-induced production of IL-1β, IL-6 and TNFα was assessed by enzyme-linked immunosorbent assay with and without anakinra in vitro, and before and after 1 month (in vivo condition) of treatment in 2 patients with SS. Spontaneous production of IL-1β, IL-6 and TNF-α by PBMCs was similar in the patients and the healthy controls and was almost undetectable. Stimulation with LPS caused a higher release of cytokines from the patients than from the healthy controls. Before in vivo anakinra start, in vitro adjunction of anakinra reduced the high LPS-induced production of IL-1β and TNFα in both patients and of IL-6 in one patient. After 1 month of treatment with anakinra, while the patients had dramatically improved, there was also a marked reduction in LPS-induced cytokines production, which was almost normalized in one patient. This study shows an abnormal LPS-induced inflammatory cytokines production in SS, which can be decreased or even normalized by in vitro and in vivo anakinra.
Highlights
Schnitzler syndrome (SS) is a rare inflammatory condition characterized by urticarial-like rash, fever and monoclonal IgM gammopathy [1]
Description of Patient 1 A 54-year-old male was referred to our institution with a 5-year history of recurrent episodes of urticarial lesions associated with fever up to 39uC
The patient experienced no episode of urticarial lesions and C-reactive protein (CRP) was normal under treatment
Summary
Schnitzler syndrome (SS) is a rare inflammatory condition characterized by urticarial-like rash, fever and monoclonal IgM gammopathy [1]. A role of interleukin (IL)-1b has been suggested [2,3,4] and IL-1 receptor antagonist anakinra was tried successfully in SS on the basis of its efficacy in treating some hereditary autoinflammatory syndromes, especially cryopyrin-associated periodic syndrome [5,6,7]. This supported the hypothesis that the inflammasome could play a crucial role in these diseases [8,9,10,11,12,13]. Before and during treatment with anakinra, IL-1b, IL-6 and TNFa plasma levels and in vitro production by PBMCs of these cytokines with and without stimulation by lipopolysaccharide (LPS) and with and without in vitro anakinra
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
