Table 1.
Demographic, organ involvement and therapy of enrolled patients.
| Clinical features | PBC–SSc patients (n = 115) | SSc patients (n = 161) | p-value |
|---|---|---|---|
| Age at Raynaud’s onset in years (mean ± SD) | 42.5 ± 16.0 | 45.5 ± 14.5 | 0.1150 |
| Age at SSc diagnosis in years (mean ± SD) | 54.5 ± 12.5 | 52.7 ± 13.1 | 0.2540 |
| Smoking (past or present), n (%) | 25 (24.7) | 34 (22.1) | 0.6819 |
| Fibrosis at HRCT, n (%) | 18 (16.8) | 29 (20.0) | 0.5390 |
| FVC <80%, n (%) | 10 (11.2) | 10 (7.3) | 0.3086 |
| FVC% (mean ± SD) | 104.2 ± 22.4 | 102.9 ± 17.2 | 0.6421 |
| DLCO <80%, n (%) | 42 (47.7) | 77 (57.5) | 0.1548 |
| DLCO% (mean ± SD) | 76.8 ± 18.7 | 76.7 ± 17.4 | 0.9581 |
| DLCO/VA <80%, n (%) | 35 (49.3) | 62 (50.0) | 0.9246 |
| DLCO/VA (mean ± SD) | 78.8 ± 18.2 | 78.9 ± 18.3 | 0.9620 |
| sPAP >45 mm Hg, n (%) | 9 (10.1) | 5 (3.8) | 0.0605 |
| sPAP (mm Hg) (mean ± SD) | 33.0 ± 15.7 | 27.7 ± 9.6 | 0.0128 |
| EF <40%, n (%) | 3 (3.2) | 3 (2.1) | 0.6865 |
| PAH, n (%) | 7 (6.6) | 3 (2.0) | 0.0984 |
| Presence of arrhythmia, n (%) | 5 (4.9) | 5 (3.3) | 0.5241 |
| Presence of conduction block, n (%) | 7 (7.1) | 7 (4.6) | 0.4128 |
| Portal hypertension | 6 (5.7) | – | – |
| DUs, n (%): | |||
| Past | 10 (8.9) | 24 (15.7) | 0.0988 |
| Current | 5 (4.4) | 13 (8.5) | 0.1912 |
| Current and past | 9 (8.0) | 15 (9.8) | 0.6048 |
| Never | 82 (78.9) | 101 (66.0) | 0.0257 |
| Gangrene, n (%) | |||
| Past | 2 (1.8) | 4 (2.6) | 1.0000 |
| Current | 1 (0.9) | 0 | 0.4226 |
| Current and past | 0 | 1 (0.7) | 1.0000 |
| Never | 101 (96.2) | 148 (96.7) | 1.0000 |
| Presence of GERD, n (%) | 56 (54.4) | 93 (64.6) | 0.1057 |
| Malabsorption, n (%) | 3 (3.0) | 6 (4.0) | 1.0000 |
| Anorectal incontinence, n (%) | 1 (1%) | 4 (2.6) | 0.6510 |
| Concomitant comorbidities, n (%): | |||
| Pernicious anaemia | 1 (0.9) | 3 (2.0) | 0.6412 |
| Graves’ or Basedow’s disease | 1 (0.9) | 1 (0.7) | 1.0000 |
| Hashimoto thyroiditis (or history of hypothyroidism) | 15 (13.5) | 7 (4.6) | 0.0250 |
| Capillaroscopic pattern, n (%): | |||
| Unspecific | 15 (16.0) | 26 (19.1) | 0.5382 |
| Early | 33 (34.7) | 55 (40.4) | 0.3797 |
| Active | 31 (33.0) | 33 (24.3) | 0.1471 |
| Late | 15 (16.0) | 23 (16.9) | 0.8481 |
| Therapy, n (%): | |||
| Immunosuppressant therapy | 20 (17.5) | 43 (28.9) | 0.0331 |
| Methotrexate | 4 | 16 | |
| Azathioprine | 3 | 4 | |
| Cyclophosphamide | 4 | 4 | |
| Chloroquine/hydroxychloroquine | 7 | 11 | |
| Mycophenolate | 1 | 5 | |
| Cyclosporine | 1 | 1 | |
| Rituximab | 1 | ||
| Leflunomide | 1 | ||
| Corticosteroids | 27 (23.9) | 28 (11.9) | 0.3288 |
| Deoxycholic acid | 84 (75.0) | – | – |
PBC: primary biliary cholangitis; SSc: systemic sclerosis; SD: standard deviation; HRCT: high-resolution computed tomography; FVC: forced vital capacity; DLCO: diffusing capacity for carbon monoxide; DLCO/VA: diffusing capacity for carbon monoxide/alveolar volume; sPAP: systolic pulmonary artery pressure; DUs: digital ulcers; EF: ejection fraction; PAH: pulmonary arterial hypertension; GERD: gastroesophageal reflux disease.
Bold values: values with statistical significance.