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Table 2 Characteristics of the cluster with Behçet’s Disease patients in Yokohama City University registry

From: Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet’s disease in Japan

Characteristics

Total (n = 657)

Cluster 1 (n = 164)

Cluster 2 (n = 140)

Cluster 3 (n = 140)

Cluster 4 (n = 146)

Cluster 5 (n = 67)

P

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

  1. ISG International Study Group, ITR-ICBD International Team for the Revision of the International Criteria for Behçet’s Disease