Variable | All Patients, n = 41 | Survived, n = 25 | Died, n = 16 | p* |
---|---|---|---|---|
Age, yrs | 50 (40–65) | 47 (38–58) | 59 (42–69) | 0.10 |
Female | 33 (80.5) | 18 (72) | 15 (93.7) | 0.12 |
BMI, kg/m2 | 23 (18.8–25.8) | 23 (17.4–26.4) | 22.9 (19.4–25) | 0.66 |
Non-SSc significant comorbidities | 10 (24.4) | 6 (24) | 4 (25) | 0.94 |
Cardiovascular diseases | 7 (17) | 5 (20) | 2 (12.5) | |
Others** | 3 (7.3) | 1 (4) | 2 (12.5) | |
Features of SSc | ||||
Time since diagnosis, mos, n = 40 | 78 (34–128) | 72 (45–105) | 97 (21–187) | 0.68 |
Type of SSc, n = 40 | 0.27 | |||
Diffuse cutaneous | 29 (72.5) | 20 (80) | 9 (56.2) | |
Limited cutaneous | 11 (27.5) | 5 (20) | 6 (37.5) | |
mRSS, n = 35 | 19 (5–30) | 20 (7–30) | 19 (3–28) | 0.60 |
Digestive involvement | 39 (95) | 25 (100) | 14 (87.5) | 0.14 |
Pulmonary involvement | ||||
NYHA stage 3–4, n = 37 | 20 (48.7) | 11 (44) | 9 (56.2) | 0.59 |
Pulmonary fibrosis on CT scan, n = 39 | 28 (71.7) | 17 (68) | 11 (68.7) | 0.48 |
DLCO, %, n = 35 | 41 (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 = 36 | 62 (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 = 38 | 12 (31.5) | 7 (28) | 5 (31.2) | 0.72 |
Renal involvement | ||||
Serum creatinine level, µmol/l, n = 39 | 84 (63–105) | 77 (52–89) | 90 (85–118) | 0.009 |
Creatinine clearance, ml/min, n = 39 | 69.7 (54.6–97.7) | 89 (61–125) | 61.4 (42–73.6) | 0.03 |
Immunological features | ||||
ANA, n = 38 | 38 (100) | 25 (100) | 13 (81.2) | 0.19 |
ACA, n = 38 | 5 (13.1) | 2 (8) | 3 (18.7) | 0.31 |
Antitopoisomerase 1 antibodies, n = 38 | 13 (34.2) | 7 (28) | 6 (37.5) | 0.32 |
Anti-RNP antibodies, n = 36 | 5 (13.8) | 4 (16) | 1 (6.2) | 0.64 |
Anti-PM/Scl antibodies, n = 37 | 1 (2.7) | 1 (4) | 0 (0) | 1 |
Anti-RNA Pol III antibodies, n = 36 | 1 (2.7) | 1 (4) | 0 (0) | 1 |
Current immunosuppressive treatment | ||||
Glucocorticoids | 27 (65.8) | 16 (64) | 11 (68.7) | 0.75 |
Immunosuppressive drugs† | 12 (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.