Table 1.
Demographic characteristics, organ involvement and clinical phenotypes in Spanish patients with IgG4-related disease
| HN (n = 26) | MS (n = 21) | ND (n = 17) | PHB (n = 14) | RA (n = 27) | Total (n = 105) | |
|---|---|---|---|---|---|---|
| Age at diagnosis, mean (s.d.), years | 50.1 (13.5)* | 55.1 (12.6) | 53.7 (15.2) | 61.0 (14.9) | 58.7 (12.3) | 55.4 (13.8) |
| Female, % (n) | 58 (15)** | 33 (7) | 29 (5) | 29 (4) | 15 (4)* | 33 (35) |
| White, % (n) | 88 (23) | 67 (14)* | 88 (15) | 86 (12) | 89 (24) | 84 (88) |
| North African/Middle East, % (n) | 4 (1) | 14 (3) | 0 (0) | 7 (1) | 0 (0) | 5 (5) |
| Hispanic, % (n) | 8 (2) | 19 (4) | 12 (2) | 7 (1) | 11 (3) | 11 (12) |
| Biopsy, % (n) | 100 (26) | 95 (20) | 100 (17) | 64 (9)*** | 93 (25) | 92 (97) |
| Pathology standard, % (n) | 81 (21) | 60 (12) | 59 (10) | 78 (7) | 80 (20) | 72 (70) |
| Elevated serum IgG4, % (n) | 19 (5)** | 62 (13)* | 41 (7) | 86 (12)*** | 26 (7) | 42 (44) |
| IPC, % (n) | 100 (26)* | 90 (19) | 76 (13) | 50 (7)*** | 93 (25) | 86 (90) |
| Highly suggestive, % (n) | 65 (17) | 62 (13) | 47 (8) | 43 (6) | 63 (17) | 58 (61) |
| Probable, % (n) | 35 (9) | 29 (6) | 29 (5) | 7 (1) | 30 (8) | 28 (29) |
| JCC, % (n) | 96 (25) | 95 (20) | 88 (15) | 100 (14) | 93 (25) | 94 (99) |
| Definite, % (n) | 23 (6) | 52 (11)* | 18 (3) | 36 (5) | 33 (9) | 32 (34) |
| Probable, % (n) | 73 (19)** | 29 (6) | 41 (7) | 21 (3) | 48 (13) | 46 (48) |
| Possible, % (n) | 0 (0)** | 14 (3) | 29 (5) | 43 (6)* | 11 (3) | 16 (17) |
| Systemic, % (n) | 35 (9) | 100 (21)*** | 53 (9) | 43 (6) | 30 (8)* | 50 (53) |
| Pancreas, % (n) | 0 (0)* | 19 (4) | 6 (1) | 71 (10)*** | 0 (0)* | 14 (15) |
| Lacrimal glands, % (n) | 19 (5)* | 14 (3) | 0 (0) | 0 (0) | 0 (0) | 8 (8) |
| Orbit, % (n) | 50 (13)*** | 24 (5) | 0 (0)* | 0 (0) | 0 (0)** | 17 (18) |
| Extraocular muscles, % (n) | 15 (4) | 14 (3) | 0 (0) | 0 (0) | 0 (0) | 7 (7) |
| Salivary glands, % (n) | 23 (6) | 43 (9)*** | 0 (0) | 7 (1) | 0 (0)* | 15 (16) |
| Pachymeninges, % (n) | 0 (0) | 24 (5)*** | 0 (0) | 0 (0) | 0 (0) | 5 (5) |
| Hypophysis, % (n) | 4 (1) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 1 (1) |
| Thyroid, % (n) | 0 (0) | 0 (0) | 18 (3)** | 0 (0) | 0 (0) | 3 (3) |
| Aorta, % (n) | 0 (0)* | 24 (5) | 0 (0) | 0 (0) | 30 (8)** | 12 (13) |
| Arteries, % (n) | 0 (0) | 10 (2) | 6 (1) | 0 (0) | 4 (1) | 4 (4) |
| Mediastinum, % (n) | 0 (0) | 19 (4)** | 6 (1) | 0 (0) | 0 (0) | 5 (5) |
| Retroperitoneum, % (n) | 0 (0)*** | 52 (11) | 29 (5) | 0 (0)** | 78 (21)*** | 35 (37) |
| Mesenterium, % (n) | 0 (0) | 10 (2) | 24 (4)* | 0 (0) | 4 (1) | 7 (7) |
| Skin, % (n) | 4 (1) | 5 (1) | 0 (0) | 0 (0) | 0 (0) | 2 (2) |
| Lymph nodes, % (n) | 8 (2) | 52 (11)*** | 24 (4) | 14 (2) | 7 (2) | 20 (21) |
| Biliary ducts, % (n) | 0 (0) | 5 (1) | 0 (0) | 29 (4)*** | 0 (0) | 5 (5) |
| Gallbladder, % (n) | 0 (0) | 0 (0) | 0 (0) | 21 (3)** | 0 (0) | 3 (3) |
| Liver, % (n) | 0 (0) | 5 (1) | 0 (0) | 14 (2)* | 0 (0) | 3 (3) |
| Lung, % (n) | 0 (0) | 33 (7)*** | 18 (3) | 0 (0) | 0 (0) | 10 (10) |
| Pleura, % (n) | 0 (0) | 14 (3) | 6 (1) | 0 (0) | 4 (1) | 5 (5) |
| Pericardium, % (n) | 0 (0) | 10 (2) | 6 (1) | 7 (1) | 0 (0) | 4 (4) |
| Kidney, % (n) | 0 (0) | 29 (6)*** | 0 (0) | 0 (0) | 4 (1) | 7 (7) |
| Breast, % (n) | 0 (0) | 0 (0) | 6 (1) | 0 (0) | 0 (0) | 1 (1) |
| Prostate, % (n) | 0 (0) | 5 (1) | 0 (0) | 0 (0) | 0 (0) | 1 (1) |
| Maxillary sinus, % (n) | 23 (6)* | 24 (5)* | 0 (0) | 0 (0) | 0 (0) | 10 (11) |
| Other organs, % (n) | 15 (4) | 24 (5)* | 6 (1) | 0 (0) | 0 (0) | 10 (10) |
Dichotomous variables were expressed as percentage (count) and continuous variables as mean (s.d.). Bivariate comparisons of continuous variables were made using Student’s t-test while bivariate comparisons of dichotomous variables were made either using χ2 test or Fisher’s exact test, as appropriate.
*
P < 0.05,
**
P < 0.01,
***
P < 0.001.
HN: head and neck; IgG4: immunoglobulin G 4; IPC: international pathology criteria; JCC: Japanese comprehensive criteria; MS: Mikulicz and systemic; ND: not defined; PHB: pancreato-hepato-biliary; RA: retroperitoneum and aorta.