Demographic features overall and by sex and race (derivation cohort)
| Sex | Race | ||||
| All (n=493) | Male (n=322) | Female (n=171) | Asian (n=208) | Non-Asian (n=285) | |
| Age at symptom onset (years, mean, SD) | 57.7 (14.5) | 59.9 (14.3) | 53.4 (14.0) | 61.2 (13.2) | 55.1 (14.9) |
| Age at diagnosis | 59.5 (14.0) | 61.7 (13.8) | 55.4 (13.5) | 62.6 (12.8) | 57.2 (14.4) |
| Time to diagnosis | 1.8 (3.4) | 1.7 (3.6) | 2.0 (2.9) | 1.4 (2.7) | 2.2 (3.7) |
| Male (n,%) | 322 (65.3%) | – | – | 126 (60.6%) | 196 (68.7%) |
| Race | |||||
| Caucasian | 198 (40.2%) | 144 (44.7%) | 54 (31.6%) | – | 198 (69.5%) |
| Asian* | 208 (42.2%) | 126 (39.1%) | 82 (47.9%) | 208 (42%) | – |
| Asian, not otherwise specified | 48 (9.7%) | 34 (10.6%) | 14 (8.2%) | 48 (9.7%) | – |
| Japanese | 105 (21.3%) | 61 (18.9%) | 44 (25.7%) | 105 (21.3%) | – |
| Chinese | 53 (10.8%) | 29 (9.0%) | 24 (14.0%) | 53 (10.8%) | – |
| Latino/Hispanic | 58 (11.8%) | 32 (9.9%) | 26 (15.2%) | – | 58 (20.4%) |
| South Asian | 14 (2.8%) | 10 (1.1%) | 4 (2.3%) | – | 14 (4.9%) |
| Black | 9 (1.8%) | 8 (2.5%) | 5 (2.9%) | – | 9 (3.2%) |
| Other | 6 (1.2%) | 2 (0.6%) | 0 (0%) | – | 6 (2.1%) |
| IgG4 concentration (mg/dL, median, IQR) | 342.5 (160–870) | 382 (177–870) | 302 (124–866) | 666 (321–1230) | 240.5 (100–505) |
| Not checked | 15 (3.0%) | 13 (4.0%) | 2 (1.2%) | 0 (0%) | 15 (5.3%) |
| Normal | 90 (18.3%) | 49 (15.2%) | 41 (24.0%) | 17 (8.2%) | 73 (25.6%) |
| >Normal < 2×ULN | 83 (16.8%) | 54 (16.8%) | 29 (17.0%) | 24 (11.5%) | 59 (20.7%) |
| >2×Normal <5×ULN | 149 (30.2%) | 98 (30.4%) | 51 (29.8%) | 67 (32.2%) | 82 (28.8%) |
| >5×ULN | 156 (31.6%) | 108 (33.5%) | 48 (28.1%) | 100 (48.1%) | 56 (19.6%) |
| Biopsy performed | 421 (85.4%) | 267 (82.9%) | 154 (90.1%) | 188 (90.4%) | 233 (81.8%) |
| Obliterative phlebitis | 91 (18.5%) | 55 (17.1%) | 36 (21.1%) | 19 (9.1%) | 72 (25.3%) |
| Storiform fibrosis | 195 (39.6%) | 126 (39.1%) | 69 (40.4%) | 57 (27.4%) | 138 (48.4%) |
| Number of organs affected (mean, SD) | 2.9 (1.8) | 2.9 (1.9) | 2.8 (1.6) | 2.9 (1.5) | 2.9 (1.9) |
| Single organ | 120 (24.3%) | 75 (23.3%) | 45 (26.3%) | 33 (15.9%) | 87 (30.5%) |
| Multiorgan (≥2 organs) | 373 (75.6%) | 247 (76.7%) | 126 (73.7%) | 175 (84.1%) | 198 (69.5%) |
| Head and Neck | 263 (53.3%) | 151 (46.9%) | 112 (65.5%) | 141 (67.8%) | 122 (42.8%) |
| Salivary gland | 186 (37.7%) | 110 (34.2%) | 76 (44.4%) | 109 (52%) | 77 (27.0%) |
| Orbital | 32 (6.5%) | 15 (4.7%) | 17 (9.9%) | 8 (3.9%) | 24 (8.4%) |
| Extraocular muscle | 20 (4.1%) | 6 (1.9%) | 14 (8.2%) | 5 (2.4%) | 15 (5.3%) |
| Lacrimal gland | 128 (26.0%) | 61 (18.9%) | 67 (39.2%) | 80 (38.5%) | 48 (16.8%) |
| Pancreato-hepatobiliary | 235 (47.7%) | 178 (55.3%) | 57 (33.3%) | 88 (42.3%) | 147 (51.6%) |
| Pulmonary | 70 (14.2%) | 51 (15.8%) | 19 (11.1%) | 30 (14.4%) | 40 (14.0%) |
| Aorta | 51 (10.3%) | 38 (11.8%) | 13 (7.6%) | 20 (9.6%) | 31 (11.0%) |
| Retroperitoneum | 78 (15.8%) | 62 (19.3%) | 16 (9.4%) | 28 (13.5%) | 50 (17.5%) |
| Renal | 77 (15.6%) | 58 (18.0%) | 19 (11.1%) | 34 (16.3%) | 43 (15.1%) |
*Two patients (0.4%) were Korean, both were male.
ULN, upper limit of normal.