most common periodic fever syndrome in childhood
The etiology is unknown.
Clinical Features
onset usually in early childhood (<5 years)
periodic autoinflammatory syndrome
attacks occurring usually every 21 to 28 days; fever lasting 5 to 7 days.
Pts report a prodrome of feeling unwell several hours before the start of an attack.
Pharyngitis and cervical adenopathy, aphthous stomatitis, abdominal pain, nausea/vomiting, arthralgia, and headaches.
Diagnosis
Diagnostic criteria - children /w typical Sx + aSx between attacks, exhibit normal growth and development, and not have cyclic neutropenia.
TREATMENT
prednisone (0.6–2 mg/kg) at the onset of Sx
Cimetidine → attacks pPx
? tonsillectomy is curative
natural course is benign; ± resolves fully into 20s