Table 1.
Characteristics of the SSc patients at baseline.
| Characteristics | All patients (n = 83;%) | ILD+ | ILD− | p* |
|---|---|---|---|---|
| (n = 39;%) | (n = 44; %) | |||
| Demographics | ||||
| Female | 79 (95) | 37 (94.87) | 42 (95.45) | 1 |
| Age in years (mean±SD) | 56.4 ± 11.3 | 56.51 ± 11.77 | 56.58 ± 10.61 | 0.979 |
| BMI (median, range) | 26 (19–40) | 28 (19–40) | 25 (20–39) | 0.06 |
| History of or current smoking attitude | 41 (49.4) | 14 (35.89) | 27 (61.36) | 0.028 |
| Disease subtype and clinical manifestations | ||||
| lcSSc | 66 (79.5) | 25 (64.1) | 41 (93.2) | |
| dcSSc | 17 (20.5) | 14 (35.9) | 3 (6.8) | 0.002 |
| Years from 1st non-RP symptom (median, range) | 12 (2–54) | 9 (2–49) | 14 (2–54) | 0.018 |
| ANA | 83 (100) | 39 (100) | 44 (100) | — |
| ACA | 33 (39.76) | 3 (7.69) | 30 (68.18) | <0.001 |
| ATA | 35 (42.2) | 32 (82.05) | 3 (6.82) | <0.001 |
| Anti-RNA pol III Ab | 2 (2.4) | 2 (5.13) | 0 | — |
| Anti-Pm-Scl Ab | 0 | 0 | 0 | — |
| Anti-fibrillarin Ab | 0 | 0 | 0 | — |
| Anti-Th/To Ab | 0 | 0 | 0 | — |
| Negative SSc-marker Ab | 13 (15.66) | 2 (5.13) | 11 (25) | 0.016 |
| RP | 83 (100) | 39 (100) | 44 (100) | — |
| History of and/or active DU and/or pitting scars | 32 (38.55) | 19 (48.72) | 13 (29.55) | 0.11 |
| mRSS (median, range) | 2 (0–28) | 3 (0–26) | 1 (0–8) | 0.001 |
| Joint/tendon involvement | 37 (44.58) | 19 (48.72) | 18 (40.91) | 0.513 |
| Muscle involvement | 3 (3.61) | 1 (2.56) | 2 (4.55) | 1 |
| Gastrointestinal involvement | 59 (71.08) | 27 (69.23) | 32 (72.72) | 0.81 |
| Kidney involvement | 0 | 0 | 0 | — |
| Circulating markers of immune system activation | ||||
| sIL2-Rα (pg/ml; median, range) | 125.4 | 161.5 | 110.1 | <0.001 |
| (59.2–394) | (78–394) | (59.2–234.5) | ||
| CCL18 (pg/ml; median, range) | 0.056 | 0.073 | 0.045 | 0.002 |
| (0.01–1) | (0.01–1) | (0.01–0.32) | ||
| Therapies | ||||
| GCs (≤10 mg/day in PDN equivalent) | 51 (61.4) | 35 (89.74) | 15 (34.1) | <0.001 |
| Previous cyclophosphamide | 39 (46.99) | 25 (64.1) | 14 (31.82) | 0.004 |
| Current azathioprine | 15 (18.07) | 8 (20.51) | 7 (15.9) | 0.776 |
| Current mycophenolate mofetil | 30 (36.14) | 23 (58.97) | 7 (15.9) | <0.001 |
| Low-dose aspirin | 83 (100) | 39 (100) | 44 (100) | — |
| CCBs | 83 (100) | 39 (100) | 39 (100) | — |
Data are number and percentages (in brackets) except where otherwise indicated.
SSc = systemic sclerosis; ILD = interstitial lung disease; SD = standard deviation; BMI = body mass index; lcSSc = limited cutaneous systemic sclerosis; dcSSc = diffuse cutaneous systemic sclerosis; RP = Raynaud phenomenon; ANA = antinuclear antibodies; ACA = anticentromere antibodies; ATA = anti-topoisomerase-I antibodies; Anti-RNA pol III = anti-RNA polymerase III; Ab = antibodies; DU = digital ulcers; mRSS = modified Rodnan Skin Score; SRC = scleroderma renal crisis; HRCT = high resolution computed tomography; sIL2-Rα = soluble interleukin-2 receptor alpha; GC = glucocorticoids; PDN = prednisone; CCBs = calcium channel blockers.
*p is ILD+ versus ILD−.