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: firstname.lastname@example.org).