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[(P)FAPA syndrome: value of cimetidine]
P Pillet1, S Ansoborlo, A Carrère
1Service de pédiatrie B, hôpital Pellegrin-Enfants, Bordeaux, France.
Unlabelled:
The (P)FAPA syndrome (periodic fever, adenitidis, pharyngitis, aphthous stomatitis) was described in 1987. The etiology of this periodic syndrome remains unknown. We report three new cases.
Case Reports:
Three girls, aged from 23 months to eight years, developed (P)FAPA. The other causes of periodic fevers were eliminated and the various treatments (antibiotics, antipyretics, nonsteroidal anti-inflammatory agents) proved ineffective. The repetition of the periodic bouts resulted in depressive disorders, absenteeism from school and a drop in weight in the youngest patient. Two of them suffered a sinusal involvement (chronic sinusitis, polyp) and had an increase in the level of immunoglobulin A. In all three cases, cimetidine at a dose of 20 mg/kg/d was well tolerated and resulted in a disappearance of the periodic fevers.
Conclusion:
Cimetidine, as an immunomodulating agent, appears to be beneficial in the in-depth treatment of (P)FAPA syndrome.