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Patients without FBLIM1 variants (62 patients)
Patients with FBLIM1 variants (18 patients)
P
Gender female
41 (66%)
11 (61%)
0,78
Median Age at onset
9.5 y (range 1.5–16)
8.9 y (range 1,5–12)
0,97
Clinical data
Fever
30 (48%)
4 (22%)
0,06
Swelling
11 (18%)
7 (38%)
0,1
Pain
60 (96%)
15 (83%)
0,07
Sites
8.9 (median)
Long bones
46 (74%)
13 (72%)
1,0
Pelvis
23 (37%)
11 (61%)
0,1
Column
22 (35%)
5 (27%)
0,78
Clavicle
16 (26%)
5 (27%)
1,0
Chest and ribs
24 (39%)
3 (16%)
0,09
Foot and hands
15 (24%)
4 (22%)
1,0
Mandible
11 (18%)
1 (5%)
0,28
Skulls
3 (5%)
0 (0%)
1,0
Skin involvements
11 (18%)
4 (22%)
0,73
Bowel involvements
3 (5%)
1 (6%)
1,0
Renal involvements
2 (3%)
0 (0%)
1,0
Laboratory data
Elevated CRP
32 (52%)
14 (77%)
0,06
Elevated ESR
41 (66%)
13 (72%)
0,78
Treatment
NSAIDs
55 (89%)
17 (94%)
0,67
Steroids
20 (32%)
6 (33%)
1,0
MTX
12 (19%)
3 (16%)
1,0
Sulfasalazine
10 (16%)
3 (16%)
1,0
Adalimumab
3 (5%)
1 (5%)
1,0
Etanercept
8 (13%)
2 (11%)
1,0
Infliximab
3 (5%)
2 (11%)
0,31
Anakinra
9 (15%)
3 (17%)
1,0
Neridronate
2 (3%)
1 (6%)
1,0
Pamidronate
29 (47%)
8 (44%)
1,0
Outcome at last follow up§
Remission without therapy
32 (52%)
5 (28%)
0,11
Remission on therapy
18 (29%)
5 (28%)
1,0
Active disease
6 (10%)
5 (28%)
0,11
