Table 1 Clinical profile of 366 patients with systemic sclerosis (SSc) at entry into the follow-up
Cases from DebrecenCases from PécsAll cases
Number of patients194*172366
Females169 (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 SSc53 (27.3%)48 (27.9%)101 (27.6%)
Limited cutaneous SSc141 (72.7%)124 (72.1%)265 (72.4%)
Anti-Scl-70 antibody68 (35.1%)66 (38.4%)134 (36.6%)
Anticentromere antibody29 (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 involvement61 (31.4%)60 (34.9%)121 (33.1%)
Oesophageal dysmotility88 (45.4%)88 (51.2%)176 (48.1%)
Oesophageal dilatation/stricture49 (25.2%)14 ( 8.1%)63 (17.2%)
Scleroderma renal crisis12 ( 6.2%)8 ( 4.6%)20 ( 5.5%)
Decreased Schirmer’s test69 (35.6%)48 (27.9%)117 (32%)
Myositis32 (16.5%)18 (10.5%)50 (13.7%)
Subcutaneous calcinosis22 (11.3%)17 ( 9.9%)39 (10.7%)
Hypo/hyperpigmentation79 (40.7%)56 (32.6%)135 (36.9%)
Teleangiectasia134 (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/hr36 (18.6%)29 (16.9%)65 (17.8%)
Malabsorption4 (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.