Current Allergy and Asthma Reports

, Volume 10, Issue 6, pp 398–404

Periodic Fever Syndromes


DOI: 10.1007/s11882-010-0141-z

Cite this article as:
Jacobs, Z. & Ciaccio, C.E. Curr Allergy Asthma Rep (2010) 10: 398. doi:10.1007/s11882-010-0141-z


The periodic syndromes represent a heterogeneous group of disorders that can be very difficult for practicing physicians to diagnosis and treat. This article presents an orderly approach to hyperimmunoglobulin D syndrome; tumor necrosis factor receptor-1 periodic syndrome; familial Mediterranean fever; periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome; and cryopyrin-associated periodic syndromes by highlighting the disease presentation, diagnosis, pathogenesis, and treatment. Recent advances are also discussed.


Periodic fever syndromePeriodic feverInflammasomeAutoinflammatory disorderHyper-IgD syndromeHIDSTumor necrosis factor receptor-1 periodic syndromeTRAPSFamilial Mediterranean feverFMFPeriodic fever with aphthous stomatitis, pharyngitis, and adenitis syndromePFAPACryopyrin-associated periodic syndromesCAPSNALP3NLRP3CryopyrinNOMIDNeonatal-onset multisystem inflammatory diseaseFamilial cold autoinflammatory syndromeFCASFamilial cold urticariaMWSMuckle-Wells syndrome

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Section of Allergy, Asthma, and ImmunologyChildren’s Mercy Hospitals and ClinicsKansas CityUSA