Comorbidity of PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenitis) patients: a case control study

To compare the long-term morbidity of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome treated by tonsillectomy (TE) in childhood to that of matched controls.

We identified 132 PFAPA patients from the medical records treated by TE in 1987-2007 in Oulu University Hospital, Finland. Altogether 119 patients participated the follow-up study and 94 were clinically examined on average 9.0 years after TE. The controls consisted of 230 randomly selected age-, sex-, and birth place-matched individuals from the Population Register Center of Finland. The patients and controls completed a detailed questionnaire about their current health and the data were compared.

Self-estimated general health was good and growth was normal among PFAPA patients and controls at long-term follow-up. There were no between-group differences in the occurrence of autoimmune or other chronic diseases. Thirty percent of the PFAPA patients and 13% of the controls reported infections as causes of hospital visits during their lifetime (p<0.001). Usage of antibiotics during lifetime was reported by 99% of the PFAPA patients and by 88% of the controls (p= 0.009). Twelve percent of PFAPA patients and 0.4% of the controls reported oral thrush in their history (p=0.003).

The health of the PFAPA patients was as good as that of healthy matched controls. Autoimmune or other chronic diseases were not more prevalent among PFAPA patients treated with TE in childhood than among controls. Respiratory infections and oral thrush were more common among the PFAPA patients than controls.