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

Excerpt

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. …