Alternative designation: FAPA
Key clinical features:
(1) periodic fevers (PF), with high fever (40°C) recurring at fixed intervals (every 2-8 weeks)
(2) aphthous stomatitis (A) may occur during the febrile episode
(3) pharyngitis (P) may occur during the febrile episode
(4) cervical adenitis (A)
The onset of fever in most patients is before 5 years of age (typical onset), but some may have an onset at an older age ("atypical").
Additional signs and symptoms that may occur during a febrile episode:
(1) malaise
(2) headache
(3) arthralgias
(4) abdominal pain
(5) vomiting
(6) hepatosplenomegaly
Laboratory changes during a febrile episode may include:
(1) an elevation in the erythrocyte sedimentation rate (ESR)
(2) a mild leukocytosis
(3) presumably an elevation in C-reactive protein (CRP)
Additional features:
(1) The patient shows a prompt drop in fever after one or two doses of prednisone.
(2) The patient fails to respond to antibiotics.
(3) The patient is clinically well between febrile episodes.
(4) Febrile episodes may last for several years and then resolve spontaneously.
(5) Some patients who meet criteria for the syndrome have responded to tonsillectomy.
Differential diagnosis:
(1) cyclic neutropenia
(2) tonsillitis
(3) hereditary periodic fever
(4) celiac disease (gluten-sensitive enteropathy)
(5) autoimmune disease or vasculitis
