PFAPA syndrome (Periodic fever, Aphthous stomatitis, Pharyngitis, Adenitis)

This paper aims to remind paediatric clinicians to suspect and confirm 'PFA PA' syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old he althy boy with atopic rhinitis and a boy aged 8 years 5 months who simultan eously had lymphocytic vasculitis syndrome treated with immunosuppressive d rugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a Medline search for articles published between 1963 an d 1998 and we then reviewed the reference lists of the articles. The Medlin e search revealed 28 cases with available clinical manifestations, manageme nt and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In t ypical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 +/- 1.4 days of fever (100%), pharyngitis (89.3%), c ervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60 .7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile interval s were 3.2 +/- 2.4 months and increased with age. The time from initial ons et to final episode was 3 years 7 months +/- 3 years 6 months. The total nu mber of episodes was 8.3 +/- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to th ose of typical PFAPA except that the age of onset was more than 5 years, an d life-threatening intestinal perforation happened once in a patient with u nderlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome i s benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other peri odic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with signific ant long-term sequelae and has a good response to steroids. One patient wit h atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to res olve atypical PFAPA.