Table 2.
Baseline clinical parameters of patients with SSc-ILD treated with cyclophosphamide: longitudinal analysis.
| Parameters | Baseline (month 0) (n = 30) | Month 1 (n = 30) | Month 6 (n = 30) | Month 12 (n = 30) | p-value (p0–1; p0–6; p0–12) |
|---|---|---|---|---|---|
| Sex: female/male, n (%) | 23 (77)/7 (23) | ||||
| Age, median (IQR) (years) | 57.7 (46.0–66.0) | ||||
| Disease duration, median (IQR) (years) | 2.8 (0.9–6.0) | ||||
| SSc subtype: dcSSc/lcSSc, n (%) | 14 (47)/16 (53) | ||||
| ANA positivity, n (%) | 25 (83) | ||||
| ACA positivity, n (%) | 2 (6) | ||||
| Anti-Scl70 positivity (%) | 16 (53) | ||||
| Raynaud’s phenomenon, n (%) | 29 (97) | ||||
| GI, n (%) | 16 (53) | ||||
| Arthralgia and/or arthritis, n (%) | 11 (37) | ||||
| ILD, n (%) | 30 (100) | ||||
| PAH, n (%) | 0 (0) | ||||
| CI, n (%) | 3 (10) | ||||
| RI, n (%) | 1 (3) | ||||
| Digital ulcers, n (%) | 6 (20) | ||||
| mRSS median (IQR) | 12.0 (4.0–19.0) | ||||
| ESSG activity score median (IQR) | 2.5 (1.0–4.0) | ||||
| Hsp90, median (IQR) (ng/mL) | 13.9 (8.6–20.6) | 18.0 (9.5–23.3) | 12.0 (8.7–21.3) | 12.5 (8.9–16.7)a | 0.683; 0.827; 0.285 |
| CRP, median (IQR) (mg/L) | 6.2 (2.7–13.4) | 6.7 (2.8–12.4) | 5.6 (2.5–14.5) | 4.7 (2.2–18.7) | 0.564; 0.117; 1.000 |
| ESR, median (IQR) (mm/h) | 14.0 (9.5–22.3) | 15.5 (10.8–25.8) | 14.5 (11.3–23.8) | 14.0 (10.0–21.0) | 0.275; 0.394; 0.467 |
| FVC, median (IQR) % predicted | 76.0 (62.8–100.4) | 74.5 (63.0–102.2) | 74.4 (60.0–98.3) | –; 0.532; 0.346 | |
| FEV1, median (IQR) % predicted | 72.4 (59.9–94.5) | 76.0 (68.0–96.5) | 69.0 (64.5–93.0) | –; 0.532; 0.467 | |
| DLCO, median (IQR) % predicted | 61.5 (46.2–75.5) | 60.0 (47.0–79.0) | 64.5 (55.5–79.0) | –; 0.297; 0.090 | |
| Current treatment: GC, n (%) | 16 (53) |
SSc systemic sclerosis, lcSSc limited cutaneous SSc, dcSSc diffuse cutaneous SSc, IQR interquartile range, ANA antinuclear antibodies, Anti-Scl-70 anti–DNA-topoisomerase I antibodies, ACA anticentromere antibodies, GI gastrointerstinal ivolvement, ILD interstitial lung disease, PAH pulmonary arterial hypertension, CI cardiac involvement, RI renal involvement, ESSG European Scleroderma Study Group, mRSS modified Rodnan skin score, CRP C-reactive protein, ESR erythrocyte sedimentation rate, FVC forced vital capacity, FEV1 forced expiratory volume in one second, DLCO diffusing capacity for carbon monoxide, GC low dose glucocorticoids (i.e. ≤ 10 mg/day of prednisone), p0–1 difference between month 0 and 1, p0–6 difference between month 0 and 6, p0–12 difference between month 0 and 12 tested by Friedman's test (for all comparisons).
aValues for plasma Hsp90 at month 12 are available only for 14 patients who underwent a 12-month cyclophosphamide treatment.