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Table 3 Characteristics of patients stratified by the presence and number of MEFV mutations

From: The association of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis

 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), years6.09 (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), months1.00 (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 (%)
 Fever28 (100%)18 (100%)11 (100%)
 Skin Rash25 (89.3%)15 (83.3%)10 (90.9%)0.870
 Joint Swelling or arthralgia28 (100%)18 (100%)11 (100%)
 Hepatomegaly10 (35.7%)4 (22.2%)2 (18.2%)0.538
 Splenomegaly15 (53.6%)4 (22.2%)5 (45.5%)0.109
 Lymphopathy15 (53.6%)9 (50.0%)6 (54.5%)1.000
 Serositis7 (25.0%)4 (22.2%)2 (18.2%)1.000
 MAS3 (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 (%)
 GC27 (96.4%)18 (100%)10 (90.9%)0.447
 MTX27 (96.4%)18 (100%)10 (90.9%)0.447
 NSAIDs26 (92.9%)16 (88.9%)11 (100%)0.661
 HCQ14 (50.0%)9 (50.0%)3 (27.3%)0.444
 LEF14 (50%)8 (44.4%)5 (45.5%)0.940
 CsA13 (46.4%)8 (44.4%)5 (45.5%)1.000
 Biologics23 (82.1%)b8 (44.4%)b8 (72.7%)0.025
  1. Abbreviation: IQR interquartile range, MAS macrophage activation syndrome, ESR erythrocyte sedimentation rate, CRP C reactive protein, SF serum ferritin, WBC white blood cells, PLT platelets, GC glucocorticoid, MTX methotrexate, NSAIDs non-steroid anti-inflammatory drugs, HCQ hydroxychloroquine, LEF leflunomide, CsA cyclosporin a
  2. aThe data of ESR and PLT are represented in mean(S.D.), while other laboratory items in median (IQR)
  3. bSignificance was found after pairwise comparison with Bofferoni correction