Characteristics | n (%) | Median (IQR) |
---|---|---|
Peripheral vasculopathy | ||
Raynaud phenomenon | 19/19 (100) | N/A |
Digital ulcers ever | 7/7 (100) | N/A |
Active digital ulcers | 5/7 (71) | N/A |
Nailfold capillaroscopy | ||
Early | 0/5 (0) | N/A |
Active | 5/5 (100) | N/A |
Late | 0/5 (0) | N/A |
Laboratory variables | ||
ANA | 12/13 (92) | N/A |
Anticentromere antibodies | 6/12 (50) | N/A |
Anti–Scl-70 | 1/8 (12.50) | N/A |
Anti–RNA-polymerase III | 1/6 (17) | N/A |
Anti-U1nRNP | 2/6 (33) | N/A |
AST elevated, U/l | 3/11 (27) | 38 (27, 89) |
Organ involvement | ||
Lung | ||
Dyspnea present | 9/9 (100) | N/A |
Pulmonary hypertensiona | 5/8 (63) | N/A |
Lung fibrosisb | 6/9 (67) | N/A |
Gastrointestinal tract | ||
Esophageal symptoms | 10/10 (100) | N/A |
Intestinal symptoms | 6/8 (75) | N/A |
Stomach symptoms | 4/7 (57) | N/A |
Melena | 5/7 (71) | N/A |
Hematemesis | 5/9 (56) | N/A |
Kidney | ||
Renal crisis | 2/6 (33) | N/A |
Liver | N/A | |
Ultrasound | ||
Liver size normal | 6/8 (75) | N/A |
Surface smooth | 3/4 (75) | N/A |
Parenchyma homogeneous | 3/4 (75) | N/A |
Focal lesions | 0/5 (0) | N/A |
Portal vein flow normal | 4/5 (80) | N/A |
Ascites | 6/8 (75) | N/A |
Splenomegaly | 9/11 (82) | N/A |
Ultrasound elastography | ||
Increased stiffness, kPa | 3/4 (75) | 14 (5, 21) |
Complications | ||
Portal hypertensionc, mmHg | 9/10 (90) | 20.5 (6, 26) |
Gastric varices, by gastroscopy | 2/6 (33) | N/A |
Esophageal varices, by gastroscopy | 10/13 (77) | N/A |
Variceal hemorrhage, by gastroscopy | 7/12 (58) | N/A |
↵a Defined as mPAP > 45 mmHg on echocardiography or > 25 mmHg on right heart catheterization.
↵b Defined as FVC < 60 or FVC < 70 and the presence of lung fibrosis on HRCT.
↵c Defined as hepatic venous pressure gradient ≥ 5 mmHg. For nominal variables, the absolute and relative frequency is shown. All other variables are presented as median with first and third quartiles. The percentages indicate positive findings in the absolute number of patients for whom the respective information was available. Italics indicate most relevant findings. Definition of items and organ manifestation are according to the EUSTAR registry11, unless otherwise specified. ANA: antinuclear antibodies; EUSTAR: EULAR Scleroderma Trials and Research group; HRCT: high-resolution computed tomography; N/A: not available; AST: aspartate aminotransferase; mPAP: mean pulmonary arterial pressure; FVC: forced vital capacity; EULAR: European League Against Rheumatism.