(P)FAPA syndrome: value of cimetidine.

The (P)FAPA syndrome (periodic fever, adenitidis, pharyngitis, aphthous sto matitis) was described in 1987 The etiology of this periodic syndrome remai ns 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 variou s treatments (antibiotics, antipyretics, nonsteroidal anti-inflammatory age nts) proved ineffective. The repetition of the periodic bouts resulted in d epressive disorders, absenteism from school and a drop in weight in the you ngest patient. Two of them suffered a sinusal involvement (chronic sinusiti s, polyp) and had an increase in the level of immunoglobulin A. In all thre e 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 benef icial in the in-depth treatment of (P)FAPA syndrome. (C) 2000 Editions scie ntifiques et medicales Elsevier SAS.