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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