Table 1.
Main demographic data and clinical and laboratory features composing the European Scleroderma Study Group index among patients enrolled in both the internal (Milan) and external (Naples) cohorts
| Milan cohort | Naples cohort | All patients | |
|---|---|---|---|
| Number of patients | 122 | 97 | 219 |
| Male/female ratio | 8/114 | 9/88 | 17/202 |
| Median age, years (range) | 52 (17–82) | 55 (19–79) | 54 (17–82) |
| Median disease duration, years (range) | 4 (0–28) | 6 (0–36) | 5 (0–36) |
| lcSSc/dcSSc | 60/62 | 72/25 | 132/87 |
| Autoantibodies | |||
| ACAs | 57 | 53 | 110 |
| Anti-Scl-70 | 58 | 22 | 80 |
| Others | 7 | 22 | 29 |
| ESSG index ≥3, n (%) | 57 (46.8) | 30 (30.9) | 87 (39.7) |
| ESSG index ≥3.5, n (%) | 49 (40.2) | 20 (20.6) | 69 (31.5) |
| Mean mRSS (range) | 5.9 (0–22) | 3.4 (0–23) | 4.6 (0–23) |
| Scleroderma, n (%) | 74 (60.7) | 17 (17.5) | 91 (41.5) |
| Change in skin involvementa, n (%) | 35 (28.7) | 23 (23.7) | 58 (26.4) |
| Ulcers, n (%) | 34 (27.9) | 11 (11.3) | 45 (20.5) |
| Change in vascular featuresa,b, n (%) | 56 (46.7) | 19 (19.5) | 75 (34.2) |
| Arthritis, n (%) | 30 (24.6) | 7 (7.2) | 37 (16.8) |
| DLCO <80%, n (%) | 86 (71.3) | 85 (87.6) | 171 (78) |
| Change in cardiopulmonary featuresa,c, n (%) | 25 (20.5) | 19 (19.5) | 44 (20) |
| ESR >30 mm/h, n (%) | 33 (27) | 35 (36) | 68 (31.1) |
| Low complement, n (%) | 15 (12.3) | 13 (13.4) | 28 (12.7) |
Abbreviations: ACAs Anti-centromere antibodies, DLCO Diffusing capacity of the lung for carbon monoxide, ESR Erythrocyte sedimentation rate, ESSG European Scleroderma Study Group, dcSSc Diffuse cutaneous systemic sclerosis, lcSSc Limited cutaneous systemic sclerosis, mRSS Modified Rodnan skin score
Data are expressed as number of patients (percent), unless otherwise specified
aDifference with respect to previous observation
bChange in vascular features was defined as subjective feeling of worsening of vascular manifestations (namely Raynaud’s phenomenon)
cChange in cardiopulmonary features was defined as subjective feeling of worsening of cardiopulmonary manifestations (namely dyspnoea)