Background:The inflammasome generates IL-1 family proteins, but its role in neutrophils is poorly understood. Results: Neutrophils store key inflammasome components in distinct intracellular compartments and release IL-1␤ and IL-18, but not IL-1␣ or IL-33. Conclusion: Neutrophils store inflammasome components in intracellular compartments. Significance: Targeting the inflammasome in neutrophils represents a future anti-inflammatory strategy.

ObjectiveTo report on the demographic data from the fi rst 18 months of enrollment to an international registry on autoinfl ammatory diseases in the context of the Eurofever project. Methods A web-based registry collecting baseline and clinical information on autoinfl ammatory diseases and related conditions is available in the member area of the PRINTO web-site. Anonymised data were collected with standardised forms.

Our data suggest that XLA might result in part from genetic inflammasome deficiency and that NLRP3 inflammasome-linked inflammation could potentially be targeted pharmacologically through BTK.

Objective. Muckle-Wells syndrome (MWS) is an inherited autoinflammatory disease resulting in exces-Methods. A multicenter cohort study of consecutive MWS patients was performed. Parameters assessed included clinical features, MWS Disease Activity Score (MWS-DAS), inflammation markers, and cytokine levels. E311K mutation-positive patients were compared with E311K mutation-negative patients. Putative risk factors for severe MWS (defined as an MWS-DAS score of >10) were assessed in univariate analyses, and significant predictors were entered into a multivariate model.Results. Thirty-two patients (15 male and 17 female) were studied. The most frequent organ manifestations were musculoskeletal symptoms and eye and skin disorders. Renal disease and hearing loss were seen in >50% of the patients. Genetically defined subcohorts had distinct phenotypes. Severe disease activity was documented in 19 patients (59%). Predictors of severe MWS identified at the time of diagnosis were female sex, hearing loss, musculoskeletal disease, increased erythrocyte sedimentation rate, and low hemoglobin level. Female sex and hearing loss remained significant after adjustment for age in a multivariate model (relative risk 1.8 and 2.6, respectively).Conclusion. MWS patients at high risk for severe disease can be identified at the time of diagnosis. Female patients presenting with hearing loss have the highest likelihood of manifesting severe MWS and should be considered a high-risk group.

MR imaging reveals musculoskeletal involvement in patients with localized scleroderma.