(P)FAPA syndrome: value of cimetidine

• Published in: Archives de pédiatrie : organe officiel de la Société française de pédiatrie Vol. 7; no. 1; pp. 54 - 57
• Main Authors: Pillet, P, Ansoborlo, S, Carrère, A, Perel, Y, Guillard, J M
• Format: Journal Article
• Language: French
• Published: France 01-01-2000
• Subjects: Child; Child, Preschool; Cimetidine - therapeutic use; Enzyme Inhibitors - therapeutic use; Female; Fever - drug therapy; Fever - etiology; Humans; Infant; Lymphadenitis - drug therapy; Lymphadenitis - etiology; Pharyngitis - drug therapy; Pharyngitis - etiology; Stomatitis, Aphthous - drug therapy; Stomatitis, Aphthous - etiology; Syndrome
• ISSN: 0929-693X
• DOI: 10.1016/S0929-693X(00)88918-6

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.
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.
Cimetidine, as an immunomodulating agent, appears to be beneficial in the in-depth treatment of (P)FAPA syndrome.