J Microbiol Immunol Infect. affecting mevalonic kinaseTNF receptor-associated periodic syndrome (TRAPS)Recurrent, 1 wkFever, abdominal pain, centrifugal rash, periorbital edema, migratory arthralgiaCorticosteroids, anakinra, etanerceptAutosomal dominant defect in gene affecting TNFRFamilial Mediterranean fever (FMF)Recurrent, 2 dSterile peritonitis with abdominal pain, monoarthritis, myalgia, erysipelas-like erythema (shins/foot)Colchicine, AnakinraaAutosomal recessive defect in em MEFV /em , affecting pyrinFamilial cold autoinflammatory syndrome (FCAS)Recurrent, 1 dArthralgia, cold-induced urticarial-like rash, conjunctivitisAnakinra, rilonacept, canakinumabAutosomal dominant defect in em NLRP3 /em , affecting cryopyrinMuckle-Wells syndrome (MWS)Recurrent, 1C2 dArthralgia, generalized urticarial-like rash, conjunctivitis, sensorineural hearing lossAnakinra, rilonacept, canakinumabAutosomal dominant defect in em NLRP3 /em , affecting cryopyrinNeonatal-onset multisystem inflammatory disease (NOMID)ChronicNeonatal onset, hepatosplenomegaly, arthropathy, generalized urticarial-like rash, uveitis, chronic aseptic DGAT1-IN-1 meningitis, sensorineural hearing lossAnakinraAutosomal dominant, often sporadic defect in em NLRP3 /em , affecting cryopyrin Open in a separate window TNF indicates tumor necrosis factor. aReported therapy needing futher investigation. CONCLUSIONS In this report we have described a subset of patients with KS who went on to develop recurrent fever syndromes, which indicates that these disorders may have a common immunologic thread, specifically related to immunodysregulation of innate immune effector mechanisms. For community pediatricians treating patients with a history of KS, the recurrence of febrile episodes should Klf4 lead to the consideration of alternative diagnoses, including recurrent fever syndromes. ACKNOWLEDGMENTS This work was supported in part by grants from the University of California San Diego Department of Pediatrics and a resident research grant from the American Academy of Pediatrics (awarded to Dr Broderick) and National Institutes of Health, National Heart, Lung, and Blood Institute grant K24 HL074864 (awarded to Dr Burns). FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. 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