ABSTRACT

The aim of this study is to evaluate the clinical and laboratory findings and results of the treatment in patients with PFAPA.

The frequency of the attacks, the age at the onset of the disease, the presence of similar symptoms in the family members, the associated symptoms found during attacks, the laboratory parameters and the results of the treatment in the patients with PFAPA were evaluated retrospectively.

Eleven patients (8 boys, 3 girls), aged between 26-60 months were evaluated. Seven of them had at least one parent with recurrent tonsillitis attacks. The complaints of the patients have begun at their ages between 8-36 months; periodic fever lasted in 3-6 days of every 3-7 weeks intervals. The pharyngitis (100%), cervical lymphadenopathy (90%), apthous stomatitis (81.8%) and exudative tonsillitis (45.45%) were found as the common symptoms. In the laboratory studies, WBC’s were 13035±1459/mm3 (11000-15890/mm3), CRP was positive, ESR’s were 32±10 mm/h (21-62 mm/h). All throat cultures were found negative for bacteria. In two patients, FMF gene mutations were found. The fever was decreased dramatically in 2-8 hours with the 1-2 mg/kg/dose oral prednisolon therapy, and it did not rise again until the next attack.

PFAPA could easily be diagnosed on patients with complaints of recurrent fever and tonsilopharyngitis by taking a good history, careful physical examination and conscious observation. By this way, early diagnosis would prevent the unnecessary usage of antibiotics.

Keywords: Periodic fever, pharyngitis, apthous stomatic, cervical lymphadenopathy, PFAPA syndrome