Table 3.

Comparison of prophylactic treatment response between patients with extreme periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (ePFAPA) and non-extreme (nPFAPA)

Variables		ePFAPA (N = 47)	nPFAPA (N = 318)	P value
Colchicine	Patients treated, n (%)	25 (53%)	61 (19%)	< 0.001
	Response (no/fewer flares), n (% of treated)	16 (64%)	43 (70%)	0.56
Cimetidine	Patients treated, n (%)	3 (6%)	6 (2%)	0.13
	Response (no/fewer flares), n (% of treated)	0 (0%)	2 (33%)	0.50
Montelukast	Patients treated, n (%)	7 (15%)	11 (3%)	< 0.001
	Response (no/fewer flares), n (% of treated)	2 (29%)	4 (36%)	> 0.099
Tonsillectomy	Patients treated, n (%)	1 (2%)	12 (4%)	> 0.99
	Response (no/fewer flares), n (% of treated)	1 (100%)	3 (25%)	0.31