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Table 1 Patient characteristics, classification, and complications in patients with YOFMF, AOFMF, and LOFMF (univariate analyses)

From: Musculoskeletal manifestations occur predominantly in patients with later-onset familial Mediterranean fever: Data from a multicenter, prospective national cohort study in Japan

Variable

YOFMF (n = 182)

AOFMF (n = 115)

LOFMF (n = 90)

p value

YOFMF vs AOFMF vs LOFMF

YOFMF vs others

Others vs LOFMF

Age at onset (years)

12.5 (6–15, n = 182)

28 (22–33, n = 115)

51 (45–61, n = 90)

NA

NA

NA

Age at diagnosis (years)

19 (12–30, n = 182)

34 (29–39, n =115)

58 (48.5–68, n = 90)

NA

NA

NA

Interval between disease onset and diagnosis (years)

7 (2–15.5, n = 182)

4 (1–11, n = 115)

2 (0.5–8, n = 90)

< 0.0001

< 0.0001

< 0.0001

Male gender

67/181 (37%)

53/115 (46%)

37/88 (44%)

0.29

0.15

0.81

Family history

51/182 (28%)

20/115 (17%)

11/90 (12%)

0.0055

0.0026

0.018

Typical FMF

111/182 (61%)

57/115 (50%)

46/90 (51%)

0.10

0.041

0.40

AA amyloidosis

2/182 (1%)

5/115 (4%)

3/90 (3%)

0.20

0.11

0.70

Autoimmune or autoinflammatory diseases

9/182 (5%)

12/115(10%)

22/90 (24%)

< 0.0001

0.0003

< 0.0001

RA

0/182 (0%)

0/115 (0%)

9/90 (10%)

< 0.0001

0.0040

< 0.0001

SLE

1/182 (1%)

1/115 (1%)

2/90 (2%)

0.43

0.63

0.23

SS

1/182 (1%)

3/115 (3%)

0/90 (0%)

0.13

0.63

0.58

PM or DM

0/182 (0%)

2/115 (2%)

1/90 (1%)

0.23

0.25

0.55

BD

0/182 (0%)

3/115 (3%)

1/90 (1%)

0.10

0.13

1.00

AOSD

0/182 (0%)

2/115 (2%)

2/90 (2%)

0.16

0.13

0.23

Others (i.e., RS3PE syndrome, Basedow disease, UC, ITP, MS, PBC, and unknown)

7/182 (4%)

1/115 (1%)

7/90 (8%)

0.040

0.98

0.054

  1. Median (interquartile range, number) or number (percentage) presented. p values established using Fisher’s exact test or Mann–Whitney U test
  2. YOFMF young-onset FMF, AOFMF adult-onset FMF, LOFMF late-onset FMF, FMF familial Mediterranean fever, NA not available, AA amyloid A, RA rheumatoid arthritis, SLE systemic lupus erythematosus, SS Sjögren’s syndrome, PM polymyositis, DM dermatomyositis, BD Behçet’s disease, AOSD adult-onset Still’s disease, RS3PE remitting seronegative symmetrical synovitis with pitting edema, UC ulcerative colitis, ITP idiopathic thrombocytopenic purpura, MS multiple sclerosis, PBC primary biliary cholangitis