The role of the tonsils in the genesis of PFAPA syndrome: a modern view and description of two clinical cases

Abstract

In ENT clinical practice, there are cases of a disease similar to those ones of chronic adenotonsillitis, but insensitive to the designated conservative therapy. This disease was first described in 1987 and is called PFAPA syndrome. Its symptoms periodicity and its regression when corticosteroids are prescribed were established. Adenotomy/adenotonsillectomy can eliminate the symptoms of the disease or significantly reduce the frequency of relapses. The histological features of the structure of the palatine and pharyngeal tonsils, as well as the characteristics of the PFAPA-syndrome, were revealed. Clinical Case Description: patient K., 3 years old, repeated cases of fever were recorded in the child from the age of 1 year 3 months. They were initially regarded as manifestations of acute respiratory viral infections, complicated by adenoiditis, pharyngitis, aphthous stomatitis and cervical lymphadenitis, with a frequency of 1.5–2 months, 6–7 episodes per year. The antiviral therapy was prescribed without any effect. A prednisolone test (1.5 mg/kg) was positive; it led to a rapid normalization of body temperature and condition improvement. The adenotonsillectomy was done. Surgical intervention and the postoperative period were uneventful. The follow-up period was without any recurrence of the disease for more than 1 year. Patient F., 6 years old, had, the recurrent (each 1.5–2 months), acute respiratory viral infections, adenotonsillitis, accompanied by lymphadenitis and febrile fever. A prednisolone test was positive. The patient was diagnosed with PFAPA syndrome by the age of 6. The adenotonsillectomy was done. No disease relapses were noted in the follow-up.