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PFAPA Syndrome

By

Apostolos Kontzias

, MD, Stony Brook University School of Medicine

Last full review/revision Mar 2020| Content last modified Mar 2020
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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 years and 5 years; 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 the most common periodic fever syndrome 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 years and 5 years) and tends to be more common among males. Recently it has been recognized in adults as well (1).

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.

General reference

  • 1. Rigante D, Vitale A, Natale MF, et al: A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol 36(2):463–468, 2017. doi: 10.1007/s10067-016-3317-7.

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, erythrocyte sedimentation rate) 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, such as a single dose of prednisone (1 to 2 mg/kg orally) or betamethasone (0.1 to 0.2 mg/kg), which, when given at the onset of an episode can dramatically abort fever attacks in a few hours, cimetidine (20 to 40 mg/kg orally once a day), 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.

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