It is the most common autoinflammatory syndrome in childhood. The onset is before 5 years of age, with attacks occurring every 21-28 days and fever lasting for 4-7 days. Phayngitis and cervical adenopathy are seen in majority of the cases, aphthous stomatitis in around 70% cases. Patients are well(with normal acute phase reactants) in- between attacks, and exhibit normal growth and development. Cyclic neutropenia and other autoinflammatory syndromes need to be excluded.
A single dose of prednisolone(0.6-2mg/kg) at the onset of symptoms usually aborts the attack. In patients needing frequent doses of corticosteroids and in those with long standing disease affecting the quality of life, tonsillectomy(with or without adenoidectomy)can be considered. Usually, the frequency and severity of attacks tends to resolve by the second decade of life.
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