Children and adolescents
Periodic fever / autoinflammatory syndromes
Stereotyped recurrent fevers: PFAPA, FMF, CAPS, TRAPS, mevalonate kinase deficiency.
What to look for
- PFAPA: fevers every 3–8 weeks, starting before 5, with aphthous ulcers, pharyngitis and cervical adenitis. The child is well between episodes.
- FMF: 1–3 day fevers with serositis. More common in Mediterranean, Middle Eastern and Armenian ancestry.
- CAPS (urticarial rash, cold-triggered), TRAPS (longer episodes, myalgia, periorbital edema), MKD (younger onset, after vaccines)
What to do
- Keep a fever diary and photograph rashes.
- Send CBC and CRP/ESR during an episode and again when well.
- PFAPA is often managed by pediatricians. A single dose of prednisone at onset usually aborts an episode. Refer if atypical or not responding.
- Colchicine is first-line for FMF (EULAR/PReS 2024). IL-1 blockade is used for colchicine-resistant FMF, CAPS, TRAPS and MKD.
About this urgency level
Specialist evaluation helps, but these are usually less time-sensitive than new inflammatory arthritis or connective tissue disease.
Timeframes are typical targets. Local pathways, capacity and clinical judgment come first.