Figure 1. Production of IL-1β, TNF-α and IL-6 by PBMCs of 2 Schnitzler syndrome (SS-1 for patient 1 and SS-2 for patient2) patients and 6 healthy subjects (HSs) in different conditions of culture.

(i) baseline: no stimulation condition of PBMCs; (ii) LPS stimulation (100 pg/ml); (iii) LPS stimulation (100 pg/ml) plus 500 ng/ml anakinra in vitro. Release of cytokine was evaluated in the supernatants collected at both 6 hours (middle gray histograms) and 16 hours (dark gray histograms) of culture conditions. Results are expressed as the mean of triplicate measures of cytokine concentration (± SD). In the SS patients, cytokine release was measured before and 1 month after in vivo anakinra treatment. In HSs, cytokine release is expressed as the mean of values (+/− SD) obtained in 6 independent evaluations.