Table 3 Characteristics of patients stratified by the presence and number of MEFV mutations
Patients without MEFV mutation (n = 28) | Patients with one heterozygous mutations (n = 18) | Patients with more than one heterozygous mutations (n = 11) | P value | |
|---|---|---|---|---|
Onset age, median (IQR), years | 6.09 (4.36) | 7.33 (5.27) | 5.33 (6.50) | 0.325 |
Male, n (%) | 10 (35.7%) | 12 (66.7%) | 6 (54.5%) | 0.121 |
Time to initiation of therapy, median (IQR), months | 1.00 (1.00) | 1.25 (2.25) | 2.00 (2.00) | 0.256 |
Number of the affected joints, median (IQR) | 5 (13) | 5 (6) | 11 (18) | 0.262 |
Manifestations, n (%) | ||||
Fever | 28 (100%) | 18 (100%) | 11 (100%) | – |
Skin Rash | 25 (89.3%) | 15 (83.3%) | 10 (90.9%) | 0.870 |
Joint Swelling or arthralgia | 28 (100%) | 18 (100%) | 11 (100%) | – |
Hepatomegaly | 10 (35.7%) | 4 (22.2%) | 2 (18.2%) | 0.538 |
Splenomegaly | 15 (53.6%) | 4 (22.2%) | 5 (45.5%) | 0.109 |
Lymphopathy | 15 (53.6%) | 9 (50.0%) | 6 (54.5%) | 1.000 |
Serositis | 7 (25.0%) | 4 (22.2%) | 2 (18.2%) | 1.000 |
MAS | 3 (10.7%) | 1 (5.6%) | 2 (18.2%) | 0.643 |
Lab examinnationa | ||||
ESR (mm/h) | 76.36(29.62) | 80.06(34.81) | 82.82(28.08) | 0.825 |
CRP (mg/L) | 132.00(112.00) | 140.00(119.50) | 72.61(83.00) | 0.182 |
SF (ng/ml) | 1832.00(3848.00) | 1361.00(1425.00) | 881.00(1329.00) | 0.184 |
WBC (×10^9/L) | 17.20(12.66) | 14.80(11.09) | 16.44(5.75) | 0.860 |
PLT (×10^9/L) | 452(134) | 462(156) | 463(136) | 0.960 |
Treatment, n (%) | ||||
GC | 27 (96.4%) | 18 (100%) | 10 (90.9%) | 0.447 |
MTX | 27 (96.4%) | 18 (100%) | 10 (90.9%) | 0.447 |
NSAIDs | 26 (92.9%) | 16 (88.9%) | 11 (100%) | 0.661 |
HCQ | 14 (50.0%) | 9 (50.0%) | 3 (27.3%) | 0.444 |
LEF | 14 (50%) | 8 (44.4%) | 5 (45.5%) | 0.940 |
CsA | 13 (46.4%) | 8 (44.4%) | 5 (45.5%) | 1.000 |
Biologics | 23 (82.1%)b | 8 (44.4%)b | 8 (72.7%) | 0.025 |