Characteristic | Median (range) |
---|---|
Sex, F/M | 6/3 |
Age, yrs | 41 (16–61) |
Disease duration*, yrs | 9 (2–12) |
Race, n | |
Caucasian | 6/9 |
Asian | 1/9 |
Black/French West Indies | 2/9 |
Antitopoisomerase antibody positivity, n | 7/9 |
Anticentromere antibody positivity, n | 0/9 |
Ever smoked cigarettes, n | 1/9 |
Performance status | 1.5 (0–4) |
Organ involvement | 0/9 |
Skin | |
mRSS | 36 (9–51) |
Lung | |
Chest radiograph | 6/9 |
HRCT | 6/9 |
VC < 70% predicted | 4/9 |
DLCO < 70% predicted | 9/9 |
PAH | 0/9 |
Systolic PAP (mm Hg) | 33 (27–40) |
Cardiovascular symptoms | |
Raynaud’s phenomenon | 9/9 |
Arterial hypertension | 0/9 |
LVEF (%) | 63 (57–70) |
Kidney** | 0/9 |
Creatinine (μmol/l) | 66 (32–80) |
Digestive tract involvement*** | 3/9 |
Muscle involvement | 0/9 |
↵* 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.