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Indexed/Abstracted in: BIOSIS Previews, EMBASE, Scopus, Emerging Sources Citation Index
Online ISSN 1827-1812
DEA - dell’Azienda ospedaliera S. Giovanni Addolorata - Roma
A syndrome of periodic fever has been identified recently by Marshall in 12 children observed at two major referral centers. The acronym used to describe the syndrome is PFAPA (for fever, aphthosis, stomatitis, pharyngitis and cervical adenitis). Attacks characterized by abrupt onset of fever, malaise, chills, aphtosis, stomatitis, pharyngitis, hedache and tender cervical adenopathy occur at 4- to 6 week intervals over periods of years. These episodes of illness resolve spontaneously in 4 to 5 day. Mild leukocytosis and elevation of the erythrocyte sedimentation rate during attacks are the only laboratory abnormalities. Affected children grow normally. In some children the syndrome resolves, whereas symptoms fn others persist for years and complications have not been described. A number of other syndromes characterized by recurrent fever have been reported and compared with PFAPA: familial Mediterranean fever, the hyper-IgD syndrome, Behçet disease, hereditary angioedema, cyclic neutropenia, familial Hibernian fever. Attacks may aborted by short courses of prednisone but do not respond to non steroidal anti-inflammatory agents. Acetaminophen and antibiotic drugs were reported to have modest therapeutic benefit Cimetidine was suggested as a prophylactic treatment for PFAPA in 1992. It appeared to be highly effective in preventing attacks in some patients. However, we cannot explain why, when cimetidine was discontinued, PFAPA syndrome did not recur. Perhaps a permanent change in these patients’ immune systems followed the 6 to 8 months of cimetidine therapy. Another possibility is spontaneous remission.