Table 1.

Baseline characteristics of patients with SSc, both in-hospital survivors and those who died. Values are n (%) or median (IQR) unless otherwise specified.

VariableAll Patients, n = 41Survived, n = 25Died, n = 16p*
Age, yrs50 (40–65)47 (38–58)59 (42–69)0.10
Female33 (80.5)18 (72)15 (93.7)0.12
BMI, kg/m223 (18.8–25.8)23 (17.4–26.4)22.9 (19.4–25)0.66
Non-SSc significant comorbidities10 (24.4)6 (24)4 (25)0.94
  Cardiovascular diseases7 (17)5 (20)2 (12.5)
  Others**3 (7.3)1 (4)2 (12.5)
Features of SSc
  Time since diagnosis, mos, n = 4078 (34–128)72 (45–105)97 (21–187)0.68
  Type of SSc, n = 400.27
    Diffuse cutaneous29 (72.5)20 (80)9 (56.2)
    Limited cutaneous11 (27.5)5 (20)6 (37.5)
  mRSS, n = 3519 (5–30)20 (7–30)19 (3–28)0.60
Digestive involvement39 (95)25 (100)14 (87.5)0.14
Pulmonary involvement
  NYHA stage 3–4, n = 3720 (48.7)11 (44)9 (56.2)0.59
  Pulmonary fibrosis on CT scan, n = 3928 (71.7)17 (68)11 (68.7)0.48
  DLCO, %, n = 3541 (25–52)40 (23–50)45 (30–55)0.37
    < 70%32 (91.4)20 (80)12 (75)0.32
    < 55%30 (85.7)19 (76)11 (68.7)0.62
  FVC, %, n = 3662 (48–77)59 (47–75)71 (38–81)0.58
    < 70%21 (58.3)15 (60)6 (37.5)0.49
    < 55%14 (42.4)10 (40)4 (25)0.72
  PAH, n = 3812 (31.5)7 (28)5 (31.2)0.72
Renal involvement
  Serum creatinine level, µmol/l, n = 3984 (63–105)77 (52–89)90 (85–118)0.009
  Creatinine clearance, ml/min, n = 3969.7 (54.6–97.7)89 (61–125)61.4 (42–73.6)0.03
Immunological features
  ANA, n = 3838 (100)25 (100)13 (81.2)0.19
  ACA, n = 385 (13.1)2 (8)3 (18.7)0.31
  Antitopoisomerase 1 antibodies, n = 3813 (34.2)7 (28)6 (37.5)0.32
  Anti-RNP antibodies, n = 365 (13.8)4 (16)1 (6.2)0.64
  Anti-PM/Scl antibodies, n = 371 (2.7)1 (4)0 (0)1
  Anti-RNA Pol III antibodies, n = 361 (2.7)1 (4)0 (0)1
Current immunosuppressive treatment
  Glucocorticoids27 (65.8)16 (64)11 (68.7)0.75
  Immunosuppressive drugs12 (29.2)7 (28)5 (31.2)1
  • * Comparisons were made between survivors and those who died.

  • ** Breast cancer in complete remission for > 5 years (n = 1), endstage renal insufficiency because of obstructive nephropathy and requiring chronic hemodialysis (n = 1), liver transplantation for fulminant viral hepatitis 15 years ago (n = 1).

  • Mycophenolate mofetil (n = 6), weekly low-dose methotrexate (n = 2), monthly pulse of cyclophosphamide (n = 2), azathioprine (n = 1), tacrolimus (n = 1). SSc: systemic sclerosis; IQR: interquartile range; BMI: body mass index; mRSS: modified Rodnan Skin score; NYHA: New York Heart Association; CT: computed tomography; FVC: forced vital capacity; PAH: pulmonary arterial hypertension; ANA: antinuclear antibodies; ACA: anticentromere antibodies; PM/Scl: polymyositis/scleroderma.