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 |