Cases from Debrecen | Cases from Pécs | All cases | |
Number of patients | 194* | 172 | 366 |
Females | 169 (87.1%) | 146 (84.9%) | 315 (86.1%) |
Age† | 41.5 (33–52.9) | 46 (38–54) | 44 (35–53) |
Disease duration (years)‡ | 15 (10–22.3) | 7 (4–13) | 12 (5–19) |
Follow-up (years, median) | 11 (7–16) | 4 (2–5) | 6 (3–12) |
Diffuse cutaneous SSc | 53 (27.3%) | 48 (27.9%) | 101 (27.6%) |
Limited cutaneous SSc | 141 (72.7%) | 124 (72.1%) | 265 (72.4%) |
Anti-Scl-70 antibody | 68 (35.1%) | 66 (38.4%) | 134 (36.6%) |
Anticentromere antibody | 29 (14.9%) | 19 ( 11%) | 48 (13.1%) |
Bibasilar lung fibrosis§ | 72 (37.1%) | 15 (8.7%) | 87 (23.8%) |
Diffuse lung fibrosis¶ | 47 (24.2%) | 70 (40.7%) | 117 (32%) |
FVC 50–80%†† | 80 (41.2%) | 120 (69.8%) | 200 (54.6%) |
FVC<50%‡‡ | 20 (10.3%) | 3 ( 1.7%) | 23 ( 6.3%) |
Cardiac involvement | 61 (31.4%) | 60 (34.9%) | 121 (33.1%) |
Oesophageal dysmotility | 88 (45.4%) | 88 (51.2%) | 176 (48.1%) |
Oesophageal dilatation/stricture | 49 (25.2%) | 14 ( 8.1%) | 63 (17.2%) |
Scleroderma renal crisis | 12 ( 6.2%) | 8 ( 4.6%) | 20 ( 5.5%) |
Decreased Schirmer’s test | 69 (35.6%) | 48 (27.9%) | 117 (32%) |
Myositis | 32 (16.5%) | 18 (10.5%) | 50 (13.7%) |
Subcutaneous calcinosis | 22 (11.3%) | 17 ( 9.9%) | 39 (10.7%) |
Hypo/hyperpigmentation | 79 (40.7%) | 56 (32.6%) | 135 (36.9%) |
Teleangiectasia | 134 (69.1%) | 80 (46.5%) | 214 (58.5%) |
Haematocrit<33% | 21 (10.7%) | 40 (22.9%) | 61 (16.7%) |
Early malignancy§§ | 6 ( 3.1%) | 10 ( 5.8%) | 16 ( 4.4) |
ESR>40 mm/hr | 36 (18.6%) | 29 (16.9%) | 65 (17.8%) |
Malabsorption | 4 (2.1%) | 9 (5.2%) | 13 (3.6%) |
*Cases starting the follow-up in Debrecen and continuing in Pécs were also included in this particular subgroup.
†Age at the onset of systemic sclerosis (median, lower-upper quartiles, year).
‡Duration of disease (median, lower-upper quartiles, year).
§Number of patients with bibasilar fibrosis on chest x ray.
¶Number of patients with diffuse fibrosis on chest x ray.
††Number of patients with 50–80% of expected FVC.
‡‡Number of patients with an FVC <50% of expected.
§§Malignancy within 4 years after the onset of SSc. For details, see Patients and methods.
ESR, erythrocyte sedimentation rate; FVC, forced vital capacity.