Atypical Familial Mediterranean Fever in a Japanese Boy with Heterozygous MEFV p.Ser503Cys Exon 5 Variant

Table 2

Profile of previously reported FMF patients with MEFV p.Ser503Cys exon 5 variants.

Patient (Ref.)	Age (yr)/sex	MEFV	FMF	Febrile attack		Other symptoms of FMF
				Frequency (mo)	Duration (day)	Ab.	Th.	Arth.	Head.	Response to colchicine
[9]	38/male	E148Q/S503C	Atyp.	<1	14	+	+	−	+	+
[10]	47/male	S503C/—	Atyp.	2	3	+	−	+	+	+
[11]	65/male	S503C/—	Atyp.	NA	NA	+	−	+	NA	+
Our case	11/male	S503C/—	Atyp.	1–3	10	+	−	−	+	+

Ab., abdominal pain; Arth., arthralgia; Atyp., atypical FMF; FMF, familial Mediterranean fever; Head., headache; mo, month; NA, not available; Ref.; reference number; Th., thoracic pain; Typ., typical FMF; yr, year.