Although, European Medicines Agency (EMA)-approved indications of anakinra currently include apart from rheumatoid arthritis only cryopyrin-associated periodic syndromes (cryopyrinopathy, CAPS), familial Mediterranean fever (FMF), and SJIA/AOSD (7), its off-label use in other conditions has been demonstrated (8–13). This evidence concerns the gene NLRP3 and familial Mediterranean fever.