PFAPA syndrome in the Czech Republic: a single-centre experience

Purpose

PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis) is an idiopathic autoinflammatory disease with the first manifestation before 5 years of age. Fever attacks lasting for 3-6 days have individual periodicity within about 3-8 week intervals. They are accompanied by individual combination of other symptoms among which culture-negative pharyngitis/tonsillitis, oral aphthae and cervical adenitis are the most common. Children are healthy and thriving in between attacks. Clinical benign course with no long-term sequelae with normal growth and development is typical for PFAPA syndrome. Fever attacks usually do not regress with antibiotic therapy, but a single prednisone dose of 1mg/kg administered at the onset of fever has a dramatic effect. Tonsillectomy appears a promising curative method for more difficult PFAPA patients.

Aim

To describe clinical and laboratory characteristics of a single-centre cohort of PFAPA patients.

Methods

Retrospective chart review (patients diagnosed 2004-2007) and prospective collection (2008-2010) of clinical and laboratory data during febrile attack and in afebrile interval.

Results

56 boys and 53 girls with PFAPA syndrome were diagnosed. Median age of the first manifestation was 20 months, median interval between attacks was 4 weeks and fever duration was 3,5 days. Patients were followed for median of 56,6 months. Fever was associated with pharyngitis/tonsillitis (87%), cervical adenitis (73%) and aphthous stomatitis (39%). Laboratory measures during the fever attack (median, range) were as follows: CRP (63,4; 12-237,4 mg/l), ESR (31; 9-60/h), WBC (14; range 3-20x10⁹/l). In all patients where follow-up values were available inflammatory parameters dropped back to normal within at least 2 weeks without fever. All patients had normal IgD levels and normal mevalonate in urine collected during the fever attack. A single prednisone dose was effective in about 90% of patients. Tonsillectomy lead to the complete remission in 12/13 children. Presence of self-limited recurrent fever with tonsillitis in early childhood of one of the parents was recorded in 45% of families.

Conclusion

PFAPA syndrome appears to be a relatively common cause of recurrent fever in early childhood in the Czech Republic. Detailed analysis of clinical and laboratory data including long-term outcomes is ongoing.

Disclosure

Petra Król: None; Marek Böhm: None; Dana Nemcová: None; Pavla Doležalová: None.