Table 3.

Preventive therapy in patients with PFAPA

Drug class/intervention	Active ingredients/treatment		Mechanism of action	Dosage	Route of administration	Efficacy	Adverse effects
Colchicine	Colchicine		IL-1β inhibition, neutrophil modulation	0.5–1 mg/day	Oral	Reduces attack frequency (especially if MEFV mutation)	Gastrointestinal discomfort
Probiotics	SSK12 Lactobacillus plantarum et paracasei		Modulates cytokines, inhibits proinflammatory bacteria	Variable	Oral	Reduces attack frequency	None reported
Vitamin D	Vitamin D		Immunomodulation, cytokine balance	400–2000 IU/day	Oral	Decreases frequency and severity of attacks in deficient patients	Hypercalcemia
Histamine receptor antagonists	Ketotifen Cimetidine		Mast cell stabilization, H1 histamine receptor inhibition H1 histamine receptor inhibition	0.08 mg/kg/day 20–40/mg/kg/day	Oral Oral	Increases attack-free interval	Restlessness, irritability, constipation Headache, dizziness, gastrointestinal discomfort
Tonsillectomy	Surgical removal of tonsils		Eliminates source of immune activation	N/A	Surgical	Complete resolution in 63–100% of cases	Surgical risks, possible recurrence of aphthous stomatitis

IL-1β interleukin-βeta, MEFV Mediterranean FeVer gene, SSK12 Streptococcus salivarius K12