Dendritic cells (DCs) are sentinels of the immune system that bridge innate and adaptive immunity. By capturing antigens in peripheral tissue, processing and presenting them with concurrent expression of costimulatory molecules and cytokine secretion they control and modulate immune reactions. Through pattern recognition receptors DCs sense molecules that are associated with infection or tissue damage frequently resulting in the formation of inflammasomes upon intracellular stimulation. The inherited autoinflammatory familial Mediterranean fever (FMF) is associated with deregulated activity of the pyrin inflammasome leading to acute inflammatory episodes. However, differentiation and function of DCs in this disease are yet unclear. Therefore, first of all, we determined DC subpopulation frequency in peripheral blood of a cohort of FMF patients. Joint evaluation without classification according to specific patient characteristics, such as mutational status, did not disclose significant differences compared to healthy controls. For the further examination of phenotype and function, we used immature and mature monocyte-derived DCs (imMo-DCs, mMo-DCs) that were generated in vitro from FMF patients. Immunophenotypic analysis of imMo-DCs revealed a significantly elevated expression of CD83, CD86, and HLA-DR as well as a significant downregulation of CD206, CD209, and GPNMB in our FMF patient group. Furthermore, FMF-imMo-DCs presented a significantly higher capacity to migrate and to stimulate the proliferation of unmatched allogeneic T cells. Finally, the transition towards a more mature and therefore activated phenotype was additionally reinforced by the fact that peripheral blood DC populations in FMF patients exhibited significantly increased expression of the costimulatory molecule CD86.This article is protected by copyright. All rights reserved.