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 |