Table 1.

Patients’ characteristics, n = 26.

Characteristicsn (%)Median (IQR)
Peripheral vasculopathy
  Raynaud phenomenon19/19 (100)N/A
  Digital ulcers ever7/7 (100)N/A
  Active digital ulcers5/7 (71)N/A
Nailfold capillaroscopy
  Early0/5 (0)N/A
  Active5/5 (100)N/A
  Late0/5 (0)N/A
Laboratory variables
  ANA12/13 (92)N/A
  Anticentromere antibodies6/12 (50)N/A
  Anti–Scl-701/8 (12.50)N/A
  Anti–RNA-polymerase III1/6 (17)N/A
  Anti-U1nRNP2/6 (33)N/A
  AST elevated, U/l3/11 (27)38 (27, 89)
Organ involvement
    Dyspnea present9/9 (100)N/A
    Pulmonary hypertensiona5/8 (63)N/A
    Lung fibrosisb6/9 (67)N/A
  Gastrointestinal tract
    Esophageal symptoms10/10 (100)N/A
    Intestinal symptoms6/8 (75)N/A
    Stomach symptoms4/7 (57)N/A
    Melena5/7 (71)N/A
    Hematemesis5/9 (56)N/A
    Renal crisis2/6 (33)N/A
      Liver size normal6/8 (75)N/A
      Surface smooth3/4 (75)N/A
      Parenchyma homogeneous3/4 (75)N/A
      Focal lesions0/5 (0)N/A
      Portal vein flow normal4/5 (80)N/A
      Ascites6/8 (75)N/A
      Splenomegaly9/11 (82)N/A
    Ultrasound elastography
      Increased stiffness, kPa3/4 (75)14 (5, 21)
  Portal hypertensionc, mmHg9/10 (90)20.5 (6, 26)
  Gastric varices, by gastroscopy2/6 (33)N/A
  Esophageal varices, by gastroscopy10/13 (77)N/A
  Variceal hemorrhage, by gastroscopy7/12 (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.