Table 2.
Treatment of acute attacks in patients with PFAPA
| Drug class | Active ingredients | Mechanism of action | Dosage | Route of administration | Efficacy | Adverse effects | ||||
|---|---|---|---|---|---|---|---|---|---|---|
| Corticosteroids |
Prednisolone Betamethasone |
Immune modulation, inflammation suppression |
1–2 mg/kg; 0.1–0.2 mg/kg (single dose); second dose if needed |
Oral |
Rapid fever resolution (85–95% of cases) |
Mood changes, sleep disturbances, increased attack frequency (25–50% of cases) | ||||
| Antipyretics | Paracetamol | Fever reduction | 15 mg/kg/dose; repeat after 4-6 h if needed | Oral | Partial and inconsistent symptom relief | Minimal with short-term use | ||||
| NSAIDs |
Ibuprofen Indomethacin |
COX inhibition |
10-15 mg/kg/dose; variable |
Oral | More effective than paracetamol but does not prevent attacks | Gastrotoxicity, nephrotoxicity | ||||
| Anti-IL-1 biologics |
Anakinra Canakinumab |
IL-1 inhibition |
1–2 mg/kg (second dose may be needed); 2 mg/kg every 8 weeks |
Subcutaneous | Rapid fever resolution in refractory cases | Injection site reactions, risk of infections | ||||
NSAIDs nonsteroidal antiinflammatory drugs, COX cyclooxygenase, IL-1 interleukin-1