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A Taste of Periodic Fever Syndromes

Koyfman, Alex MD*; Lovallo, Emily MD; Hazen, Melissa M. MD‡§; Chiang, Vincent W. MD‡∥

doi: 10.1097/PEC.0b013e318298df8b
CME Review Article

Periodic fevers are acquired or inherited disorders of innate immunity, which were first described in the 1940s. The patients are typically young at onset and have regularly recurring fevers for a few days to a few weeks with systemic inflammatory symptoms that are interrupted by symptom-free periods. There is a variety of clinical manifestations including gastrointestinal complaints, myalgias, arthralgias, and rash. A differential diagnosis in these patients may include recurrent infections, other inflammatory disorders, and neoplastic disease. This clinical review focuses on a sample of autoinflammatory disorders including familial Mediterranean fever, tumor necrosis factor receptor 1–associated periodic syndrome, hyperimmunoglobulinemia D syndrome, the cryopyrin-associated periodic syndrome, and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. We review the basics, pertinent clinical and laboratory features, and management of each entity.

Resident (Koyfman), *Department of Emergency Medicine, University of Illinois College of Medicine at Peoria, OSF Saint Francis Medical Center, Peoria, IL; Resident (Lovallo), †Department of Emergency Medicine, Alameda County Medical Center and Highland General Hospital, Oakland, CA; and Rheumatology attending (Hazen), Divisions of ‡General Pediatrics, §Rheumatology, and EM attending (Chiang), ∥Emergency Medicine, Children’s Hospital Boston, Boston, MA.

The authors and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interest in, any commercial organizations pertaining to this educational activity.

Reprints: Alex Koyfman, MD, Emergency Medicine Residency, OSF St Francis Medical Center, 530 N.E. Glen Oak Avenue Peoria, IL 61637 (e-mail:

© 2013 Lippincott Williams & Wilkins, Inc.