|
Clinical symptoms used in clustering
|
|
Oral ulcer, n (%)
|
653 (99.4)
|
164 (100.0)
|
138 (98.6)
|
140 (100.0)
|
144 (98.6)
|
67 (100.0)
|
0.265
|
|
Skin involvement, n (%)
|
585 (89.0)
|
164 (100.0)
|
130 (92.9)
|
140 (100.0)
|
93 (63.7)
|
58 (86.6)
|
< 0.001
|
|
Eye involvement, n (%)
|
392 (59.7)
|
78 (47.6)
|
48 (34.3)
|
76 (54.3)
|
146 (100.0)
|
44 (65.7)
|
< 0.001
|
|
Genital ulcer, n (%)
|
474 (72.1)
|
164 (100.0)
|
105 (75.0)
|
140 (100.0)
|
21 (14.4)
|
44 (65.7)
|
< 0.001
|
|
Arthritis, n (%)
|
346 (52.7)
|
164 (100.0)
|
94 (67.1)
|
0 (0.0)
|
57 (39.0)
|
31 (46.3)
|
< 0.001
|
|
Gastrointestinal involvement, n (%)
|
113 (17.2)
|
0 (0.0)
|
106 (75.7)
|
0 (0.0)
|
0 (0.0)
|
7 (10.4)
|
< 0.001
|
|
Vascular involvement, n (%)
|
55 (8.4)
|
0 (0.0)
|
54 (38.6)
|
0 (0.0)
|
0 (0.0)
|
1 (1.5)
|
< 0.001
|
|
Neurological involvement, n (%)
|
67 (10.2)
|
0 (0.0)
|
1 (0.7)
|
0 (0.0)
|
0 (0.0)
|
66 (98.5)
|
< 0.001
|
|
Criteria and HLA typing, smoking, and hospitalization rates
|
|
Sex: female/male, n/available data number (%)
|
372/285 (56.6/43.4)
|
124/40 (75.6/24.4)
|
80/60 (57.1/42.9)
|
90/50 (64.3/35.7)
|
46/100 (31.5/68.5)
|
32/35 (47.8/52.2)
|
< 0.001
|
|
Age at onset (years, mean ± SD)
|
36.58 ± 12.29
|
37.37 ± 12.25
|
35.41 ± 13.41
|
33.59 ± 10.25
|
40.48 ± 12.84
|
34.79 ± 10.43
|
< 0.001
|
|
Observation period (years, mean ± SD)
|
13.72 ± 11.79
|
16.33 ± 12.07
|
13.72 ± 12.52
|
13.27 ± 12.53
|
10.16 ± 9.44
|
16.08 ± 10.70
|
< 0.001
|
|
Pathergy test, n (%)
|
74/170 (43.5)
|
27/66 (40.9)
|
7/23 (31.8)
|
21/34 (61.8)
|
13/32 (40.6)
|
6/16 (37.5)
|
0.174
|
|
HLA-B51, n (%)
|
214/449 (47.7)
|
58/114 (50.9)
|
29/88 (33.0)
|
49/94 (52.1)
|
49/98 (50.0)
|
29/55 (52.7)
|
0.047
|
|
Fulfilling ISG criteria, n (%)
|
583 (88.7)
|
164 (100.0)
|
107 (76.4)
|
140 (100.0)
|
113 (77.4)
|
59 (88.1)
|
< 0.001
|
|
Fulfilling ITR-ICBD criteria, n (%)
|
645 (98.2)
|
164 (100.0)
|
128 (91.4)
|
140 (100.0)
|
146 (100.0)
|
67 (100.0)
|
< 0.001
|
|
Hospitalization, n/available data number (%)
|
239/522 (45.8)
|
35/115 (30.4)
|
69/97 (71.1)
|
43/128 (33.6)
|
49/125 (39.2)
|
43/57 (75.4)
|
< 0.001
|
|
Blindness, n/available data number (%)
|
98/582 (16.8)
|
10/141 (7.1)
|
8/124 (6.5)
|
22/133 (16.5)
|
34/123 (27.6)
|
24/61 (39.3)
|
< 0.001
|
|
Death caused by BD, n/available data number (%)
|
3/520 (0.6)
|
0/114 (0.0)
|
2/96 (2.1)
|
1/128 (0.8)
|
0/125 (0.0)
|
0/57 (0.0)
|
0.230
|
|
Treatments
|
|
Colchicine, n/available data number (%)
|
375/522 (71.8)
|
73/115 (63.5)
|
77/97 (79.4)
|
83/128 (64.8)
|
96/125 (76.8)
|
46/57 (80.7)
|
0.009
|
|
Glucocorticoids, n/available data number (%)
|
236/522 (45.2)
|
40/115 (34.8)
|
65/97 (67.0)
|
44/128 (34.4)
|
42/125 (33.6)
|
45/57 (78.9)
|
< 0.001
|
|
Maximum dose of prednisolone (mg/day, mean ± SD)
|
11.75 ± 17.76
|
5.64 ± 11.15
|
18.98 ± 19.77
|
6.81 ± 12.71
|
8.16 ± 14.24
|
31.81 ± 23.64
|
< 0.001
|
|
Methylprednisolone pulse therapy, n/available data number (%)
|
36/522 (6.9)
|
2/115 (1.7)
|
9/97 (9.3)
|
2/128 (1.6)
|
4/125 (3.2)
|
19/57 (33.3)
|
< 0.001
|
|
Immunosuppressants, n/available data number (%)
|
203/522 (38.9)
|
17/115 (14.8)
|
71/97 (73.2)
|
30/128 (23.4)
|
47/125 (37.6)
|
38/57 (66.7)
|
< 0.001
|
|
Biologics, n (%)
|
95 (14.5)
|
9 (5.5)
|
23 (16.4)
|
15 (10.7)
|
34 (23.3)
|
14 (20.9)
|
< 0.001
|
|
Time from diagnosis to biologics (years, mean ± SD)
|
6.22 ± 8.52
|
4.86 ± 6.90
|
7.47 ± 10.95
|
10.07 ± 10.20
|
2.96 ± 4.46
|
8.54 ± 8.42
|
0.043
|
