Table 1.
Disease-specific characteristics of patients with SSc
| Characteristic | SSc patients (n = 19) |
|---|---|
| Female, n (%) | 12 (63) |
| Presence of RP, n (%) | 19 (100) |
| Time since onset of RP, median (IQR), years | 10.1 (2.4, 21.8) |
| Time since onset of first non-RP symptom, median (IQR), years | 7.5 (1.8, 19.5) |
| History of, n (%) | |
| Digital ulceration | 6 (32) |
| GORD | 17 (90) |
| ILD | 8 (42) |
| Palpitations | 9 (47) |
| Standard of care tested cardio-pulmonary profile, mean (s.d.), % | |
| Forced vital capacity | 92 (25) |
| Total lung capacity | 92 (20) |
| DLCO | 63 (16) |
| DLCO/VA | 78 (17) |
| Electrocardiogram, n (%) | |
| Rhythm | 19 (100) sinus rhythm |
| Axis | 2 (11) LAD |
| Block | 1 (5) incomplete RBBB, 1 (5) LBBB, 1 (5) left anterior fascicular block |
| LV ejection fraction % on echocardiogram, mean (s.d.) | 56 (9) |
| RV–RA gradient on echocardiogram, mean (s.d.), mmHga | 22 (6) (n = 12) |
| CV risk profile | |
| Smoking status: never/ex/current, n (%) | 8 (42)/11 (58)/0 (0) |
| Hypertension, n (%) | 0 (0) |
| Hypercholesterolaemia, n (%) | 0 (0) |
| FH premature CVDb, n (%) | 2 (11) |
| Serology and acute phase, n (%) | |
| ANA positive | 18 (95) |
| ACA positive | 6 (32) |
| Scl70 positive | 5 (26) |
| CRP (normal range <5), median (IQR), mg/l | 0 (0.0, 1.3) |
| Cardiovascular biomarkers and lipid profile | |
| hs-TnI, mean (s.d.), ng/l | 76 (137) |
| NT-proBNP, mean (s.d.), ng/l | 145 (130) |
| CK, mean (s.d.), IU/l | 141 (148) |
| Glucose, mean (s.d.), mmol/l | 4.8 (0.5) |
| Total cholesterol, mean (s.d.), mmol/l | 4.9 (1.0) |
| HDL-C, mean (s.d.), mmol/l | 1.5 (0.4) |
| LDL-C, mean (s.d.), mmol/l | 2.8 (0.8) |
| TC/HDL-C ratio, mean (s.d.) | 3.7 (1.3) |
| Triglycerides, mean (s.d.), mmol/l | 1.6 (0.8) |
Many values missing due to poor tricuspid regurgitation/image quality.
Defined as first degree relative with a history of CVD when 60 years old or younger if female, and 55 years old or younger if male. CK: creatine kinase; CV: cardiovascular; CVD: cardiovascular disease; DLCO: diffusing capacity of the lungs for carbon monoxide; DLCO/VA: DLCO adjusted for volume; GORD: gastro-oesophageal reflux disease; FH: family history of; HDL-C: high density lipoprotein cholesterol; hs-TnI: high-sensitivity troponin I; ILD: interstitial lung disease; IQR: interquartile range; LAD: left axis deviation; LBBB: left bundle branch block; LDL-C: low density lipoprotein cholesterol; LV: left ventricular; NT-proBNP: N-terminal pro-brain natriuretic peptide; RBBB: right bundle branch block; RP: Raynaud’s phenomenon; RV-RA: right ventricular-right atrial; Scl70: anti-topoisomerase antibody.