Table 1.
Characteristics of SSc patients by DU status^
| Patient characteristics (n = 1085) | DU | No DU | p value |
|---|---|---|---|
| Mean ± SD or n(%) | Mean ± SD or n(%) | ||
| Number of patients | 527 (48.6%) | 558 (51.4%) | |
| Demographics | |||
| Age on SSc onset*, years | 43.6 ± 13.9 | 48.8 ± 14.0 | < 0.001 |
| Disease duration at recruitment, years | 12.2 ± 10.5 | 10.1 ± 9.9 | 0.001 |
| Gender female | 432 (81.9%) | 494 (88.5%) | 0.002 |
| Male | 95 (18.0%) | 64 (11.5%) | 0.002 |
| Disease subtype limited disease subtype | 342 (65.%) | 455 (81.8%) | < 0.001 |
| Diffuse disease subtype | 184 (34.9% | 101 (18.2%) | < 0.001 |
| Caucasian ethnicity | 476 (95.9%) | 502 (93.7%) | 0.09 |
| Follow-up, years | 5.2 ± 2.5 | 4.7 ± 2.4 | 0.001 |
| Alive at censorship | 363 (78.1%) | 412 (83.1%) | 0.05 |
| Autoantibody profile** | |||
| Anti-centromere pattern ANA (n = 1059) | 213 (41.2%) | 268 (49.5%) | 0.007 |
| Scl 70 + ve (n = 1046) | 96 (18.9%) | 50 (9.3%) | < 0.001 |
| RNA polymerase III + ve (n = 693) | 57 (16.9%) | 42 (11.8%) | 0,05 |
| Clinical manifestations*** | |||
| Telangiectasia ever | 504 (95.6%) | 452 (81.0%) | < 0.001 |
| Calcinosis ever | 299 (56.7%) | 179 (32.1%) | < 0.001 |
| Joint contractures | 321 (60.9%) | 153 (27.4%) | < 0.001 |
| GIT involvement | 478 (90.7%) | 456 (81.7%) | < 0.001 |
| SSc renal crisis | 19 (3.6%) | 14 (2.5%) | 0.293 |
| PAH# | 87 (16.5%) | 65 (11.7%) | 0.02 |
| ILD | 185 (53.1%) | 149 (26.7%) | 0.003 |
| Co-morbidities | |||
| CVA | 33 (6.3%) | 30 (5.4%) | 0.53 |
| Diabetes mellitus | 37 (7.0%) | 52 (9.3%) | 0.17 |
| PVD | 26 (4.9%) | 6 (1.1%) | < 0.001 |
| Smoking history (current or ever) | 275 (52.2%) | 275 (52.2%) | 0.19 |
| Medications | |||
| Calcium channel blocker (CCB) | 414 (78.6%) | 311 (55.7%) | < 0.001 |
| PDE5 inhibitor | |||
| Sildenafil | 99 (18.8%) | 46 (8.2%) | < 0.001 |
| Tadalafil | 11 (2.1%) | 10 (1.8%) | 0.724 |
| Endothelial receptor antagonist (ERA) | |||
| Ambrisentan | 30 (5.7%) | 18 (3.2%) | 0.05 |
| Bosentan | 100 (18.9%) | 81 (14.5%) | 0.05 |
| Macitentan | 17 (3.2%) | 13 (2.3%) | 0.37 |
| Iloprost | 148 (28.1%) | 10 (1.8%) | < 0.001 |
| Topical vasodilator | 71 (13.5%) | 10 (1.8%) | < 0.001 |
| HRQoL | |||
| Physical component score (PCS) | 36.1 ± 10.4 | 39.2 ± 11.2 | < 0.001 |
| Mental component score (MCS) | 46.3 ± 12.8 | 46.1 ± 13.9 | 0.84 |
Abbreviations: DU digital ulceration, PAH pulmonary arterial hypertension, ILD interstitial lung disease, GIT gastrointestinal tract, CVA cerebrovascular accident, PVD peripheral vascular disease, ACA anticentromere, Scl-70 antitopoisomerase-1, RNAP anti-RNA polymerase III, CCB calcium channel blockers, ERAs endothelial receptor antagonists, PDE5 phosphodiesterase-5 inhibitors
Health-related quality of life (HRQoL), was defined using the SF-36 study short form which provides a score range from 0 to 100.·Scores below 50 indicate worse HRQoL than the population normative score and every 10 points indicates 1 standard deviation.·These scores can be summarized into the physical component score (PCS) and mental component score (MCS), scores below 50 indicate worse HRQoL than the population normative score and every 10 points indicates 1 standard deviation
^DU status defined as the physicians reported presence of any history of DU
*SSc onset defined as the first symptom of SSc (Raynaud phenomenon or other) *disease duration defined as from first non-Raynaud’s disease manifestation
**n denotes the number of patients who underwent the bloods test thereby determining if they were positive or negative
***clinical manifestations defined as present if ever present from SSc diagnosis
#PAH diagnosed on right heart catheterization (RHC) according to international criteria [11]