Table 1.
Characteristics | N*. with Available Data n (%) | SSc Patients Presented as Frequency (Percent), or Median (Interquartile Range) |
---|---|---|
Demographics | ||
Sex, female | 372 (100) | 308 (82.8) |
Age, years | 372 (100) | 59.0 (50.0; 68.0) |
BMI, kg/m2 | 372 (100) | 24.5 (21.9; 28.0) |
Disease characteristics | ||
Cutaneous subset, diffuse | 372 (100) | 73 (19.6) |
Disease duration # | 372 (100) | |
≤3 years | 92 (24.7) | |
>3 years | 280 (75.3) | |
Antibody status | ||
Anti-nuclear antibodies | 372 (100) | 365 (98.1) |
Anti-centromere | 197 (53) | |
Anti-topoisomerase I | 82 (22) | |
Anti-RNA polymerase III | 12 (3.2) | |
Anti RNP | 11 (3) | |
Organ involvement | ||
Interstitial lung disease | 371 (99) | 146 (39.4) |
Limited ILD £Extensive ILD £ | 103 (71) | |
42 (29) | ||
FVC% | 371 (99) | 104.0 (91.0; 115.0) |
DLCO% | 363 (97) | 70.0 (59.0; 81.0) |
mRSS | 372 (100) | 2.5 (1.0; 6.0) |
Gastrointestinal tract | 372 (100) | 311 (83.6) |
Pulmonary hypertension | 372 (100) | 12 (3.2) |
Renal crisis | 364 (97) | 2 (0.5) |
Digital ulcers | 370 (99) | 168 (45.4) |
Raynaud phenomenon | 372 (100) | 370 (99.5) |
Overlap syndrome | 372 (100) | 129 (34.7) |
Current treatment | ||
Glucocorticoids | 193 (52) | 64 (33.2) |
Immunosuppressive drugs $ | 193 (52) | 48 (24.9) |
Values are presented as frequency (percent), median (interquartile range). N* number, SSc systemic sclerosis, BMI body mass index. # Disease duration defined from the first symptom outside the Raynaud phenomenon, FVC% forced vital capacity (% predicted value), DLCO% carbon monoxide lung diffusion capacity (% predicted value). RNA Ribonucleic acid. RNP Ribonucleoprotein. $ Immunosuppressive drugs used were Cyclophosphamide, mycophenolate mofetil, azathioprine, methotrexate, anti-calcineurin, intravenous immunoglobulins, rituximab, anti TNFalpha, tocilizumab (no patients received tocilizumab). £ Limited ILD: Limited involvement of less than 10% of pulmonary parenchyma; Extensive ILD: reaching more than 30% of pulmonary parenchyma. In patients with HRCT extent of 10–30% (termed indeterminate disease), an FVC threshold of 70% was an adequate prognostic substitute, according to Goh et al. ATS journal, 2008. During follow-up, 32 patients (8.6%) died.