Article Figures & Data
Figures
- Figure 1.
Short-term and longterm vital status. *One additional patient lost to followup. **Three additional patients lost to followup. ICU: intensive care unit.
- Figure 2.
Survival according to the requirement of endotracheal intubation and mechanical ventilation. Log-rank test p < 0.001. ICU: intensive care unit.
Tables
- 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.
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.
- Table 2.
Intensive care management of patients with SSc, including in-hospital survivors and those who died. Data were available for all 41 patients. Values are n (%) or median (IQR) unless otherwise specified.
Variable All Patients, n = 41 Survived, n = 25 Died, n = 16 p* Time from hospital to ICU admission, days 1 (0–4) 1 (0–2) 1 (0–11) 0.58 Reasons for ICU admission 0.42 Acute respiratory failure 27 (65.8) 16 (64) 11 (68.7) Acute renal failure 6 (14.6) 5 (20) 1 (6.2) Others 8 (19.5) 4 (16) 4 (25) Main diagnosis 0.07 Pulmonary infection** 14 (34.1) 7 (28) 7 (43.7) Cardiovascular complications† 8 (19.5) 3 (12) 5 (31.2) Scleroderma renal crisis 7 (17) 5 (20) 2 (12.5) Others‡ 12 (29.2) 10 (40) 2 (12.5) Admission characteristics Mean arterial pressure 88 (77–104) 92 (81–105) 79 (71–98) 0.03 Glasgow coma scale 15 (15–15) 15 (15–15) 14 (7–15) 0.01 Serum protein level, g/l 69 (59–73) 70 (63–76) 59 (52–68) 0.002 Serum creatinine level, µmol/l 88 (56–160) 72 (52–204) 101 (90–170) 0.10 Serum bilirubin level, µmol/l 7.5 (4–11.7) 6 (3–9) 12 (5–27) 0.007 Admission severity scores SAPS2, points 38 (23–53) 26 (22–36) 55 (41–89) < 0.001 SOFA, points 4 (4–7) 4 (2–5) 7 (5–11) < 0.001 Life-sustaining interventions Noninvasive ventilation 13 (31.7) 6 (24) 7 (43.7) 0.30 Invasive mechanical ventilation 13 (31.7) 2 (8) 11 (68.7) < 0.001 Vasopressors 13 (31.7) 1 (4) 12 (75) < 0.001 Renal replacement therapy 12 (29.2) 3 (12) 9 (56.2) 0.002 In-ICU length of stay, days 3 (2–7) 2 (2–3) 5 (3–11) 0.005 ↵* Comparisons were made between survivors and those who died.
↵** Microbiological documentation in 5 patients: Streptococcus pneumoniae (n = 2), Escherichia coli and Klebsiella pneumoniae (n = 1), Pseudomonas aeruginosa (n = 1), Pneumocystis jirovecii (n = 1).
↵† Cardiovascular complications: acute pulmonary edema (2), myocarditis (2), pulmonary embolism (2), out-of-hospital cardiac arrest (1), acute right ventricular failure (1).
↵‡ Others: acute respiratory failure without any definite diagnosis (5), drug-induced pneumonitis (1), DRESS syndrome (1), meningeal hemorrhage (1), acute cytolytic hepatitis (1), pneumothorax (1). SSc: systemic sclerosis; IQR: interquartile range; ICU: intensive care unit; SAPS2: Simplified Acute Physiology Score 2; SOFA: Sequential Organ Failure Assessment; DRESS: Drug Reaction with Eosinophila and Systemic Symptoms.
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