Table 1:
Demographics and Features of the IgG4-Related Disease Cohort
| Overall | |
|---|---|
| N | 231 |
| Age at Diagnosis (mean, SD) | 59.5 (13.7) |
| Male (N, %) | 150 (65%) |
| Race | |
| White | 173 (75%) |
| Asian | 32 (14%) |
| Black | 10 (4%) |
| Native-American | 1 (<1%) |
| Unknown / Other | 15 (6%) |
| Ethnicity | |
| Non-Hispanic | 185 (80%) |
| Hispanic | 29 (13%) |
| Unknown / Other | 17 (7%) |
| Selected Organ Involvement | |
| Head and Neck | 137 (59%) |
| Dacryo- or Sialoadenitis | 113 (49%) |
| Lacrimal glands | 50 (22%) |
| Salivary glands | 98 (42%) |
| Orbital (non-lacrimal) | 34 (15%) |
| Other head and neck | 109 (47%) |
| Lymph nodes | 63 (27%) |
| Pulmonary | 44 (19%) |
| Aorta | 21 (9%) |
| Retroperitoneum | 41 (18%) |
| Pancreato-hepatobiliary | 78 (34%) |
| Renal | 47 (20%) |
| Laboratory Results (median, IQR) | |
| IgG4 Concentration (n=228) | 142.4 (53.2, 390.8) |
| % Ever Elevated^ | 168 (73%) |
| Eosinophil Concentration (n=193) | 0.20 (0.10, 0.40) |
| % Elevated^ | 40 (21%) |
| IgE Concentration (n=192) | 104.0 (25.0, 284.5) |
| % Elevated^ | 98 (51%) |
| Erythrocyte Sedimentation Rate (n=159) | 24.0 (10.0, 45.0) |
| % Elevated^ | 66 (42%) |
| C-Reactive Protein (n=161) | 3.8 (1.3, 9.1) |
| % Elevated^ | 53 (33%) |
| C3 (n=191) | 115.0 (85.0, 145.0) |
| % Low C3 | 23 (12%) |
| C4 (n=193) | 23.0 (12.0, 31.0) |
| % Low C4 | 32 (17%) |
^
Elevated refers to serum IgG4 concentration > the assay’s upper limit of normal, serum IgE concentration ≥ 100 IU/mL, eosinophils ≥ 0.5 109/L, ESR > 30 mm/hr, and CRP > 7 mg/L