OR6-003 – Prospective evaluation of PFAPA patients

Results In our cohort (148 males and 120 females) fever began at 26.2 + 24 months of age. 8% of the patients had an onset after the fifth year of life, but all other Thomas criteria were met. A family history was present in 39.6% of patients. Mean duration of PFAPA episodes was 4 + 1.6 days, and a mean interval between episodes 27.9 + 11 days. Most common symptoms with fever were pharyngitis (95.5%), cervical adenitis (63.8%), stomatous aphtosis (38.4%), abdominal pain (32%). Prodromes, such as irritability, nausea and headache were present in 10% of patients. All patients received treatment with oral steroids, using a single administration of 1 mg/kg of prednisone or prednisone equivalent, the first day of fever. In all patients steroids were effective and only 13 % of them experienced a free-interval shortening, without the perceived need to stop the steroids for this reason. There was no difference in the studied parameters between the population who experienced a free-interval shortening and the population in which this event was not registered. In 144 children resolution occurred, in 58 % of children spontaneously and in 42 % after tonsillectomy. Mean disease duration was 40 + 63 months, medium age at resolution 67.7 + 66 months. Tonsillectomy was efficacious in 60/62 patients. Mutation analysis for FMF, HIDS, and TRAPS in the latter two patients were negative. The tonsillectomy was done after a mean period of 36 months from disease onset. At multivariate regression analysis disease resolution was independently associated to age onset (b = 1.011 95 % CI 1.000-1.022, p = 0.05) and to tonsillectomy (b = 0.022 95 %CI 0.005-0.092 p = 0.001).