Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden

Table 6 Characteristics of PFAPA patients and frequency of symptoms during fever episodes in different studies

Authors	Year	N	Sex M/F %	Age of onset^a (years)	Time between episodes^a (weeks)	Duration of episodes^a (days)	Pharyngitis (%)	Cervical adenitis (%)	Aphthous stomatitis (%)	Abdominal pain (%)	Diarrhea (%)	Skin rash (%)	Conjunctivitis (%)
Marshall et al. [1]	1987	12	58/42	N/A	4.5	5	75	67	75	50	N/A	8	N/A
Thomas et al. [9]	1999	94	55/45	2.8	4	4.8	65	77	67	45	30	15	N/A
Padeh et al. [24]	1999	28	71/29	4.2	5.1	4.3	100	100	68	18	N/A	N/A	N/A
Tasher et al. [25]	2006	54	61/39	1.9	3.7	5.3	96	61	39	65	13	4	N/A
Feder et al. [26]	2010	105	62/38	3.3	4.3	4.1	85	62	38	41	N/A	N/A	N/A
Forsvoll et al. [18]	2013	46	70/30	0.9	3.5	4	approx. 80	approx. 90	approx. 50	approx. 40	N/A	N/A	N/A
Krol et al. [27]	2013	125	50/50	1.9	4	3.5	91	78	41	23	5	3	3
Hofer et al. [28]	2014	301	53/47	1.7	4	4	90	78	57	45	16	13	5
Perko et al. [29]	2015	81	63/37	2.1	4	4.2	98	94	56	51	22	12	N/A
Batu et al. [30]	2016	131	65/35	1.8	3.7	4	96	53	43	46	11	5	N/A
Amarilyo et al. [31]	2020	303	60/40	3.0	4.7/3.5/3.9 ^b	4.8/4.3/3.9 ^b	88/96/90 ^b	47/51/47 ^b	29/38/33 ^b	24/55/64 ^b	N/A	N/A	N/A
Results in this study	2022	336	54/46	2.0	4	4.5	94	81	43	33	2	5	1

^aMean or median, depending on what was reported
^bThis study investigates differences between ethnic groups and the numbers refer to the three reported groups Non-Mediterranean/Mediterranean/Multiethnic

ISSN: 1546-0096