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Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study

Authors


Correspondence

Jostein A Førsvoll, Department of Pediatrics, Stavanger University Hospital, Postboks 8100, 4068 Stavanger, Norway.

Tel.: +47 51518228 |

Fax: +47 51519940 |

Email: fojo@sus.no

Abstract

Aim

To describe the incidence, epidemiology, clinical presentation and clinical outcome of children with the syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) in a population-based study.

Methods

In a prospective population-based study, all children in South Rogaland, Norway, diagnosed with PFAPA during 2004–2010 were evaluated clinically, and parents were interviewed systematically. A follow-up interview was performed for all patients.

Results

A total of 46 children (32 boys; p = 0.011) were diagnosed with PFAPA. We calculated an incidence of 2.3 per 10 000 children up to 5 years of age. The median age of onset was 11.0 months (quartiles: 5.0, 14.8). Nearly 37 children were followed until resolution. In 17 of these, a tonsillectomy was performed with prompt resolution of PFAPA in all. The median age of spontaneous resolution was 60.2 months (range 24–120) and in children with tonsillectomy 50.9 months (range 15–128).

Conclusion

The incidence of PFAPA was 2.3 per 10 000 children up to 5 years of age. In the majority of cases, onset of symptoms may be during the first year of life.

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