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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)

From: Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients

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

  1. 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
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Pediatric Rheumatology

ISSN: 1546-0096