Familial Mediterranean fever is no longer a rare disease in Japan

Table 11 Clinical features of FMF patients with different numbers of MEFV mutations

	No. of MEFV mutations
Clinical manifestations	≥2	0 or 1
	n = 117	n = 75	p
Male/female	52/65	31/44	0.671
Age at onset (years), mean ± SD	28.3 ± 15.2	33.7 ± 19.2	0.097
Fever	113 (96.6 %)	71 (94.7 %)	0.383
Frequencies of febrile attack (per month), mean ± SD	1.11 ± 0.97	0.97 ± 0.83	0.438
Duration of fever attack (days), mean ± SD	3.1 ± 2.1	4.6 ± 5.6	0.201
Abdominal pain	51 (43.6 %)	26 (34.7 %)	0.218
Thoracic pain	36 (30.8 %)	15 (20.0 %)	0.099
Arthritis	69 (59.0 %)	39 (52.0 %)	0.342
Erysipelas-like erythema	17 (14.5 %)	17 (22.7 %)	0.150
AA amyloidosis	7 (6.0 %)	0	0.029
Family history of periodic fever	31 (26.5 %)	8 (10.7 %)	0.008
Rheumatic diseases	18 (15.4 %)	8 (10.7 %)	0.351

Values are shown as n (%) unless otherwise indicated. AA amyloid A, FMF familial Mediterranean fever

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