PFAPA syndrome (the syndrome of Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis) is an idiopathic autoinflammatory condition. The current pharmacological treatment includes episodic application of corticosteroids and many other options for the prophylaxis of flares (e.g. anakinra, ketofifen, oral probiotics). We present our group of patients and the efficacy of different prophylactic options.
We present our group of patients with PFAPA syndrome, which were diagnosed according to valid clinical criteria in our Centre for Periodic Fever Syndromes in Martin, Slovakia.
144 patients with this diagnosis were identified, 74 boys and 70 girls, aged 4.01±2.78 years. The mean age of symptoms’ onset was 2.26±1.92 years. Positive family history was found in 21.36% of patients. During attacks 100% of patients had fever; pharyngitis and cervical adenitis was present in 96% of patients, 50% of patients had aphthous stomatitis. All of our patients yielded elevation of inflammatory parameters (CRP, serum amyloid A, IL-6) during attacks. All of the patients were treated episodically with prednisone with excellent clinical response. For the prohylaxis of febrile episode we used ketotifen with positive effect observed in 63% of patients. Specific oral probiotic strain (Streptococcus salivarius K12) was used in 22 patients with recurrent aphthous stomatitis with good outcome in 5 of 22 patients. Tonsillectomy was indicated in 14% of patients with excellent clinical response.
In our cohort of PFAPA patients we showed unique characteristics of this patients and confirmed the positive effect of new treatment possibilities, e.g. ketotifen or oral probiotic strain.