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Masson Regnault, Marie; Frouin, Eric; Jéru, Isabelle; Delwail, Adriana; Charreau, Sandrine; Barbarot, Sébastien; Néel, Antoine; Masseau, Agathe; Puéchal, Xavier; Kyndt, Xavier; Gayet, Stephane; Lifermann, François; Asli, Bouchra; Balguerie, Xavier; Blanchard-Delaunay, Claire; Aubin, François; Rizzi, Rita; Rongioletti, Franco; Boyé, Thierry; Gusdorf, Laurence; Bessis, Didier; Morel, Franck; Hainaut, Ewa; Lipsker, Dan; Lecron, Jean-Claude
Frontiers in immunology, 11/2020, Volume: 11Journal Article
Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by urticarial exanthema, bone and joint alterations, fever and monoclonal IgM gammopathy. Overactivation of the interleukin(IL)-1 system is reported, even though the exact pathophysiological pathways remain unknown. To determine v cytokine profiles of Peripheral Blood Mononuclear Cells (PBMCs) from SchS patients prior to treatment and after initiation of anti-IL-1 therapy (anakinra). The sera cytokine profile was studied in parallel. We collected blood samples from thirty-six untreated or treated SchS. PBMCs were cultured with and without LPS or anti-CD3/CD28. Cytokine levels were evaluated in serum and cell culture supernatants using Luminex technology. Spontaneous TNFα, IL-6, IL-1β, IL-1α, and IL-1RA release by PBMCs of SchS patients were higher than in controls. LPS-stimulation further induced the secretion of these cytokines. In contrast, after T-cell stimulation, TNFα, IL-10, IFNγ, IL-17A, and IL-4 production decreased in SchS patients compared to healthy controls, but less in treated patients. Whereas IL-1β serum level was not detected in most sera, IL-6, IL-10, and TNFα serum levels were higher in patients with SchS and IFNγ and IL-4 levels were lower. Of note, IL-6 decreased after treatment in SchS ( = 0.04). Our data strengthen the hypothesis of myeloid inflammation in SchS, mediated in particular by IL-1β, TNFα, and IL-6, associated with overproduction of the inhibitors IL-1RA and IL-10. In contrast, we observed a loss of Th1, Th2, and Th17 cell functionalities that tends to be reversed by anakinra.
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