Table 1.
General characteristics of patients with primary Sjögren’s syndrome with and without associated cancer
| pSS Patients with solid cancer and/or hemopathy n = 55 | pSS patients with solid cancer without hemopathy n = 40 | pSS patients with pSS and hemopathy only n = 8 | pSS patients with more than one cancer and/or hemopathy n = 12 | pSS patients without history of solid cancer and/or hemopathy n = 110 | |||||
|---|---|---|---|---|---|---|---|---|---|
| n (%) | p value* | n | p value* | n (%) | p value* | n (%) | p value* | n (%) | |
| Female (n, %) | 52 (94.5%) | 1.000 | 37 (92.5%) | 1.000 | 8 (100,0%) | 0.456 | 11 (91.7%) | 0.588 | 102 (92.7%) |
| Age at pSS diagnosis (mean ± SD) | 58.4 (± 10.4) | 0.005 | 59.3 (± 9.36) | 0.004 | 50,6 (± 10,4) | 0.028 | 58 (± 17.5) | 0.364 | 52.3 (± 13.9) |
| Follow-up (mean ± SD) (years) | 10.5 (± 10.1) | 0.178 | 9.9 (± 8.4) | 0.170 | 16.5 (± 15.9) | 0.148 | 10.3 (± 9.7) | 0.663 | 7.9 (± 8.3) |
| MSGB positivity (n, %) | 43 (86.0%) | 0.427 | 30 (75.0%) | 0.352 | 8 (100,0%) | 0.361 | 8 (66.7%) | 0.287 | 93 (84.5%) |
| Anti-Ro/SSa and/or Anti La/SSb positivity (n, %) | 21 (38.2%) | 0.284 | 15 (37.5%) | 0.301 | 4 (50,0%) | 1.000 | 8 (66.7%) | 0.363 | 50 (45.5%) |
| Sicca syndrome (n, %) | 42 (91.3%) | 0.006 | 33 (82.5%) | 0.011 | 5 (62,5%) | 0.574 | 10 (83.3%) | 0.280 | 80 (72.7%) |
| Constitutional symptoms (n, %) | 11 (20.0%) | 0.611 | 4 (10.0%) | 0.321 | 4 (50,0%) | 0.043 | 3 (25.0%) | 0.440 | 15 (13.6%) |
| Joint involvement (n, %) | 40 (72.7%) | 0.690 | 32 (80.0%) | 0.213 | 5 (62,5%) | 0.700 | 7 (58.3%) | 0.514 | 76 (69.1%) |
| Lymphadenopathy (n, %) | 15 (27.3%) | < 0.001 | 4 (10.0%) | 0.454 | 5 (62,5%) | < 0.001 | 6 (50.0%) | < 0.001 | 5 (4.5%) |
| Cutaneous activity (n, %) | 15 (27.3%) | 0.020 | 9 (22.5%) | 0.127 | 4 (50,0%) | 0.017 | 3 (25.0%) | 0.208 | 15 (13.6%) |
| Pulmonary activity (n, %) | 11 (20%) | 0.133 | 5 (12.5%) | 0.802 | 3 (37,5%) | 0.068 | 4 (33.3%) | 0.056 | 11 (10.0%) |
| Renal activity (n, %) | 4 (7.3%) | 0.067 | 1 (2.5%) | 0.519 | 1 (12,5%) | 0.156 | 3 (25.0%) | 0.005 | 2 (1.8%) |
| Peripheral neurological system (n, %) | 9 (16.4%) | 0.159 | 7 (17.5%) | 0.143 | 0 (0,0%) | 0.384 | 3 (25.0%) | 0.114 | 10 (9.1%) |
| Autoimmune cytopenia (n, %) | 4 (7.3%) | 1. 000 | 2 (5.0%) | 1.000 | 0 (0,0%) | 0.454 | 2 (16.7%) | 0.234 | 7 (6.3%) |
| Hypocomplementemia (n, %) | 7 (14.6%) | 0.048 | 2 (5.0%) | 0.649 | 2 (25,0%) | 0.057 | 3 (25.0%) | 0.027 | 4 (3.6%) |
| Cryoglobulinemia (n, %) | 7 (16.3%) | 0.026 | 2 (5.0%) | 0.581 | 2 (25,0%) | 0.041 | 3 (25.0%) | 0.010 | 2 (1.8%) |
| Hypergammaglobulinemia (n, %) | 20 (36.4%) | 0.511 | 12 (30.0%) | 0.907 | 4 (50,0%) | 0.431 | 4 (33.3%) | 1.000 | 31 (28.2%) |
| Monoclonal gammopathy (n, %) | 14 (25.5%) | 0.031 | 5 (12.5%) | 0.870 | 4 (50,0%) | 0.015 | 6 (50.0%) | 0.004 | 11 (10.0%) |
| RF + (n, %) | 22 (40.0%) | 0.003 | 12 (30.0%) | 0.110 | 6 (75,0%) | 0.001 | 4 (33.3%) | 0.242 | 23 (20.9%) |
| ANA + (n, %) | 44 (80.0%) | 0.197 | 32 (80.0%) | 0.249 | 7 (87,5%) | 0.435 | 3 (25.0%) | 1.000 | 78 (70.9%) |
| ESSDAI max (mean ± SD) | 12.0 (± 13.0) | < 0.001 | 7.4 (± 7.1) | 0.136 | 20.6 (± 10.8) | < 0.001 | 20.2 (± 19.9) | 0.002 | 5.5 (± 5.0) |
| Corticosteroids (n, %) | 26 (47.3%) | 0.361 | 17 (42.5%) | 0.752 | 5 (62,5%) | 0.27 | 7 (58.3%) | 0.215 | 42 (38.2%) |
| Hydroxychloroquine (n, %) | 33 (60.0%) | 0.550 | 26 (65.0%) | 0.094 | 5 (62,5%) | 0.717 | 6 (50.0%) | 0.976 | 54 (49.1%) |
| Methotrexate (n, %) | 13 (23.6%) | 0.628 | 9 (22.5%) | 0.769 | 2 (25,0%) | 0.667 | 5 (41.7%) | 0.138 | 19 (17.3%) |
| Rituximab (n, %) | 9 (16.4%) | 0.167 | 3 (7.5%) | 1.000 | 4 (50,0%) | 0.007 | 3 (25.0%) | 0.117 | 8 (7.2%) |
| Immunosuppressive therapy (CYC or AZA) (n, %) | 2 (3.6%) | 0.710 | 2 (5.0%) | 1.000 | 0 (0,0%) | 0.454 | 0 (0%) | 1.000 | 7 (6.3%) |
| Hypertension (n, %) | 28 (50.9%) | 0.082 | 19 (47.5%) | 0.226 | 4 (50,0%) | 0.467 | 9 (75.0%) | 0.012 | 41 (37.3%) |
| Cardiopathy (n, %) | 12 (21.8%) | 0.001 | 6 (15.0%) | 0.067 | 3 (37,5%) | 0.011 | 3 (25.0%) | 0.033 | 8 (7.3%) |
| Diabetes (n, %) | 10 (18.2%) | 0.056 | 8 (20.0%) | 0.042 | 1 (12,5%) | 0.504 | 4 (33.3%) | 0.023 | 9 (8.2%) |
| Autoimmune disease (n, %) | 13 (23.6%) | 0.685 | 10 (25.0%) | 0.842 | 1 (12,5%) | 0.675 | 4 (33.3%) | 0.732 | 31 (28.2%) |
| Family history of breast cancer (n, %) | 7 (20.6%) | 0.026 | 6 (15.0%) | 0.022 | 0 (0,0%) | 1.000 | 1 (8.3%) | 0.284 | 3 (2.7%) |
| Smoking exposure (n, %) | 12 (28.6%) | 0.616 | 11 (27.5%) | 0.433 | 1 (12,5%) | 1.000 | 2 (16.7%) | 0.637 | 24 (21.8%) |
| Excessive alcohol consumption (n, %) | 4 (10.8%) | 0.091 | 3 (7.5%) | 0.131 | 0 (0.0%) | 1.000 | 1 (8.3%) | 0.209 | 3 (2.7%) |
MSBG, minor salivary gland; RF, rheumatoid factor; ANA, antinuclear antibodies; ESSDAI, EULAR Sjögren’s syndrome disease activity index; CYC, cyclophosphamide; AZA; azathioprine
% were adjusted according to available data.
*univariate analyses were performed against the control group of 110 pSS patients without cancer, p value < 0.05