[Periodic fever in children: keep in mind the PFAPA syndrome].
The autoinflammatory diseases should be considered in the differential diagnosis of recurrent fever in childhood. These diseases are characterized by inflammatory episodes without an evident cause. Some of these diseases, like the Familial Mediterranean Fever, have a genetic origin and need a chronic treatment to avoid severe complications on the long term. PFAPA syndrome is the most frequent cause of recurrent fever and is diagnosed based on unspecific criteria. The treatment is still controversial. One dose of Prednisone is able to interrupt the flare and tonsillectomy may induce a remission in the majority of the cases.
Hofer
M
M
Unité d'immuno-allergologie et rhumatologie pédiatrique, Service de pédiatrie, CHUV, Lausanne. Michael.hofer@chuv.ch
Rossetti
G
G
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001171:Arthritis, Juvenile / Q000175:diagnosis; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis; D007072:Immunoglobulin D / Q000276:immunology; D008199:Lymphadenitis / Q000175:diagnosis*; D010612:Pharyngitis / Q000175:diagnosis*; D011241:Prednisone / Q000627:therapeutic use; D018124:Receptors, Tumor Necrosis Factor / Q000276:immunology; D013281:Stomatitis, Aphthous / Q000175:diagnosis*; D013577:Syndrome; D014068:Tonsillectomy
D000893:Anti-Inflammatory Agents; D007072:Immunoglobulin D; D018124:Receptors, Tumor Necrosis Factor; D011241:Prednisone