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PFAPA Syndrome

By Apostolos Kontzias, MD, Assistant Professor of Medicine and Director, Autoinflammatory Clinic, Cleveland Clinic Foundation

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PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is a periodic fever syndrome that typically manifests between ages 2 yr and 5 yr; it is characterized by febrile episodes lasting 3 to 6 days, pharyngitis, aphthous ulcers, and adenopathy. Etiology and pathophysiology are undefined. Diagnosis is clinical. Treatment can include glucocorticoids, cimetidine, and, rarely, tonsillectomy.

PFAPA syndrome is a relatively common periodic fever among children. Although genetic causes have not been determined, this syndrome tends to be grouped with hereditary fever syndromes. It typically starts in early childhood (between ages 2 yr and 5 yr) and tends to be more common among males.

Febrile episodes last 3 to 6 days and recur about every 28 days. The syndrome causes fatigue, chills, and occasionally abdominal pain and headache, as well as fever, pharyngitis, aphthous ulcers, and lymphadenopathy. Patients are healthy between episodes, and growth is normal.

Diagnosis

  • Clinical evaluation

Diagnosis of PFAPA syndrome is based on clinical findings, which include the following:

  • 3 febrile episodes, lasting up to 5 days and occurring at regular intervals

  • Pharyngitis plus adenopathy or aphthous ulcers

  • Good health between episodes and normal growth

Acute-phase reactants (eg, C-reactive protein, ESR) are elevated during a febrile episode but not between episodes. Neutropenia or other symptoms (eg, diarrhea, rash, cough) are not present; their presence suggests a different disorder. Specifically, cyclic neutropenia needs to be ruled out.

Treatment

  • Sometimes glucocorticoids, cimetidine, and/or tonsillectomy

Treatment of PFAPA syndrome is optional; it can include glucocorticoids, cimetidine, and, rarely, tonsillectomy. Other drugs such as anakinra have been tried with some success in refractory cases. Patients tend to outgrow this syndrome without sequelae.