Table 1 Baseline characteristics and phenotypic distribution of the IgG4-RD study cohort
All | Pancreato-Hepato-Biliary | Retroperitoneum and Aorta | Head and Neck-Limited | Mikulicz’ and Systemic | p-value | |
|---|---|---|---|---|---|---|
N (%) | 79 (100) | 18 (22.8) | 18 (22.8) | 23 (29.1) | 20 (25.3) | |
Male subjects, n (%) | 53 (67.1) | 15 (83.3) | 12 (66.7) | 10 (43.5) | 16 (80.0) | 0.024 |
White, n (%) | 63 (79.7) | 17 (94.4) | 15 (83.3) | 15 (65.2) | 16 (80.0) | 0.135 |
Age at diagnosis, years (SD) | 57.8 (14.2) | 62.3 (14.9) | 63.8 (8.8) | 48.8 (16.0) | 58.6 (10.3) | 0.002 |
Serum IgG4, g/L (SD) (n = 73) a | 6.06 (6.02) | 5.35 (4.66) | 2.5 (1.6) | 4.6 (4.4) | 11.6 (7.7) | < 0.001 |
Elevated baseline serum IgG4, n (%) (n = 76) a | 59/76 (77.6) | 13/18 (72.2) | 11/16 (68.8) | 17/22 (77.3) | 18 (90.0) | 0.425 |
CRP, mg/dL (SD) | 12.9 (25.6) | 4.6 (6.6) | 37.0 (44.7) | 8.3 (15.6) | 6.0 (7.5) | < 0.001 |
ESR, mm/h (SD) | 32.3 (29.6) | 17.7 (11.1) | 56.4 (35.4) | 23.3 (25.7) | 32.6 (25.9) | 0.001 |
Biopsy, n (%) b | 62 (78.5) | 17 (94.4) | 6 (33.3) | 22 (95.7) | 17 (85.0) | < 0.001 |
Number of involved organs (SD) | 3.7 (2.1) | 3.3 (1.5) | 2.2 (1.6) | 2.9 (1.4) | 6.2 (1.5) | < 0.001 |
≥ 2 involved organs, n (%) | 68 (86.1) | 17 (94.4) | 11 (61.1) | 20 (87.0) | 20 (100) | 0.003 |
RCD, (%) | < 0.001 | |||||
Any | 72 (91.1) | 18 (100) | 12 (66.7) | 23 (100) | 19 (95.0) | |
Definite | 39 (49.4) | 13 (72.2) | 2 (11.1) | 11 (47.8) | 13 (65.0) | |
Probable | 14 (17.7) | 4 (22.2) | 2 (11.1) | 7 (30.4) | 1 (5.0) | |
Possible | 19 (24.1) | 1 (5.6) | 8 (44.4) | 5 (21.7) | 5 (25.0) | |
ACR/EULAR classification criteria, n (%) | 57 (72.2) | 18 (100) | 5 (27.8) | 14 (60.9) | 20 (100) | < 0.001 |