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). The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.