Table 3.
Characteristics of the cluster with Behçet’s disease patients in MHLW registry
| Total (n = 6754) | Cluster A (n = 1131) | Cluster B (n = 876) | Cluster C (n = 689) | Cluster D (n = 805) | Cluster E (n = 1369) | Cluster F (n = 799) | Cluster G (n = 1085) | P | |
|---|---|---|---|---|---|---|---|---|---|
| Clinical symptoms used in clustering | |||||||||
| Oral ulcer, n (%) | 6336 (93.8) | 1050 (92.8) | 867 (99.0) | 361 (52.4) | 805 (100.0) | 1369 (100.0) | 799 (100.0) | 1085 (100.0) | < 0.001 |
| Skin involvement, n (%) | 5483 (81.2) | 493 (43.6) | 755 (86.2) | 177 (25.7) | 805 (100.0) | 1369 (100.0) | 799 (100.0) | 1085 (100.0) | < 0.001 |
| Eye involvement, n (%) | 2344 (34.7) | 87 (7.7) | 115 (13.1) | 538 (78.1) | 805 (100.0) | 0 (0.0) | 799 (100.0) | 0 (0.0) | < 0.001 |
| Genital ulcer, n (%) | 4242 (62.8) | 575 (50.8) | 202 (23.1) | 212 (30.8) | 0 (0.0) | 1369 (100.0) | 799 (100.0) | 1085 (100.0) | < 0.001 |
| Arthritis, n (%) | 3311 (49.0) | 461 (40.8) | 484 (55.3) | 192 (27.9) | 390 (48.4) | 1369 (100.0) | 415 (51.9) | 0 (0.0) | < 0.001 |
| Gastrointestinal involvement, n (%) | 906 (13.4) | 879 (77.7) | 0 (0.0) | 27 (3.9) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | < 0.001 |
| Vascular involvement, n (%) | 148 (2.2) | 0 (0.0) | 121 (13.8) | 27 (3.9) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | < 0.001 |
| Neurological involvement, n (%) | 350 (5.2) | 0 (0.0) | 289 (33.0) | 61 (8.9) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | < 0.001 |
| Other characteristics | |||||||||
| Sex: female/male, n (%) | 3990/2764 (59.1/40.9) | 654/477 (57.8/42.2) | 427/449 (48.7/51.3) | 299/390 (43.4/56.6) | 295/510 (36.6/63.4) | 1032/337 (75.4/24.6) | 479/320 (59.9/40.1) | 804/281 (74.1/25.9) | < 0.001 |
| Age at onset (years, mean ± SD) | 36.68 ± 14.23 | 39.91 ± 17.73 | 38.54 ± 14.46 | 38.78 ± 15.14 | 36.82 ± 13.09 | 35.58 ± 12.64 | 34.45 ± 12.00 | 33.38 ± 12.37 | < 0.001 |
| Observation period (years, mean ± SD)* | 4.14 ± 7.62 | 4.05 ± 7.71 | 4.18 ± 7.28 | 6.32 ± 9.95 | 3.82 ± 6.91 | 3.07 ± 6.06 | 6.19 ± 9.90 | 2.87 ± 5.57 | < 0.001 |
| Pathergy test, n/available data number (%) | 1361/4225 (32.2) | 225/706 (31.9) | 171/536 (31.9) | 117/431 (27.1) | 149/404 (29.6) | 295/860 (34.3) | 175/508 (34.4) | 229/680 (33.7) | 0.099 |
| HLA-B51, n/available data number (%) | 1389/3141 (44.2) | 172/513 (33.5) | 237/492 (48.0) | 172/358 (48.0) | 221/413 (53.5) | 244/599 (40.7) | 164/321 (51.1) | 179/443 (40.4) | < 0.001 |
| Fulfilling ISG criteria, n (%) | 4980 (73.7) | 405 (35.8) | 333 (38.0) | 185 (26.9) | 805 (100.0) | 1368 (99.9) | 799 (100.0) | 1085 (100.0) | < 0.001 |
| Fulfilling ITR-ICBD criteria, n (%) | 5586 (82.7) | 657 (58.1) | 456 (52.1) | 416 (60.4) | 805 (100.0) | 1368 (99.9) | 799 (100.0) | 1085 (100.0) | < 0.001 |
| Recent hospitalization, n/available data number (%)** | 1556/6002 (25.9) | 400/971 (41.2) | 299/771 (38.8) | 144/625 (23.0) | 77/738 (10.4) | 271/1195 (22.7) | 121/728 (16.6) | 244/974 (25.1) | < 0.001 |
ISG International Study Group, ITR-ICBD International Team for the Revision of the International Criteria for Behçet’s Disease
*“Observation period” indicates the period from disease onset to application of specific disease by the Japanese government
**“Recent hospitalization” indicates episode of admission less than 6 months per patients