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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