Table 6.

Comparison of the interval between flares after steroid treatment between patients with non-extreme periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (nPFAPA), extreme responder PFAPA(erPFAPA) and extreme PFAPA upon presentation (ePFAPA upon presentation)

Variables	nPFAPA (N = 318)	erPFAPA (N = 25)	ePFAPA upon presentation (N = 22)	P value
Increased episode frequency with steroids, n (%)	74/183 (40%)	20/24 (83%)	9/16 (56%)	0.002
Intervals between flares after initiation of abortive treatment with corticosteroids (weeks), Median (IQR), N = 192	2.5 (1.93–4)	1.0 (1.0–1.5)	1.0 (1.0–1.38)	< 0.001

The nPFAPA group comprised 318 patients. The erPFAPA groupcomprised 25 patients. The ePFAPA group upon presentation comprise 22 patients. The proportions and the percentages reflect the missing data