Table 1.

Baseline clinical and serologic characteristics of the patients with SSc (n = 9) treated by autologous hematopoietic stem cell transplant according to the ISAMAIR protocol5 and followed at least 1 year after transplant with repeated CT scans.

CharacteristicMedian (range)
Sex, F/M6/3
Age, yrs41 (16–61)
Disease duration*, yrs9 (2–12)
Race, n
  Caucasian6/9
  Asian1/9
  Black/French West Indies2/9
Antitopoisomerase antibody positivity, n7/9
Anticentromere antibody positivity, n0/9
Ever smoked cigarettes, n1/9
Performance status1.5 (0–4)
Organ involvement0/9
  Skin
    mRSS36 (9–51)
  Lung
    Chest radiograph6/9
    HRCT6/9
    VC < 70% predicted4/9
    DLCO < 70% predicted9/9
    PAH0/9
    Systolic PAP (mm Hg)33 (27–40)
  Cardiovascular symptoms
    Raynaud’s phenomenon9/9
    Arterial hypertension0/9
    LVEF (%)63 (57–70)
  Kidney**0/9
    Creatinine (μmol/l)66 (32–80)
  Digestive tract involvement***3/9
  Muscle involvement0/9
  • * Time elapsed between first non-Raynaud’s phenomenon and autologous hematopoietic stem cell transplant.

  • ** 24-h urinary protein > 0.5 g or serum creatinine > 120 μmol/l.

  • *** Serum albumin < 25 g/l or weight loss > 10% body weight in the preceding year. mRSS: modified Rodnan skin score; HRCT: high resolution computed tomography of the chest; VC: slow inspiratory vital capacity; DLCO: diffusing capacity for carbon monoxide; PAH: pulmonary arterial hypertension as defined by systolic pulmonary arterial hypertension (PAP) > 40 mm Hg; LVEF: left ventricle ejection fraction as measured by cardiac echography or multiple gated acquisition CT scan.