Baseline characteristics of the cohort of patients with systemic sclerosis (SSc). The data are derived from the cohort of patients with SSc registered with EBMT for stem cell or bone marrow transplant. All patients with a minimum of three months' follow up data or who had died were included. In patients of disease duration >3 years, haemopoietic stem cell transplantation was performed owing to involvement of the vital organs Table 1(A) Basic demographic data and the duration of follow up
Demographic characteristic | Cohort data | |
Total patient number | 41 | |
Age (years), median (range) | 41 (11.5–58.1) | |
Sex (F:M) | 34:7 | |
Disease pattern | 37 diffuse:4 limited | |
Disease duration (months), median (range) | 29 (2–158) | |
Duration of follow up (months), median (range) | 12 (3–55) | |
Table 1(B) Disease severity criteria for this group of patients | ||
Disease severity criterion | No/No evaluable* | % |
Skin score >50% maximum† | 20/33 | 61 |
Skin score % of maximum, median (range) | 56.9 (21–92) | |
FVC‡ <70% of body mass related mean | 18/36 | 50 |
Tlco ‡<70% of body mass related mean | 23/35 | 66 |
Creatinine clearance | ||
<100 ml/min | 13/27 | 48 |
<70 ml/min | 1/27 | 4 |
Scl-70 antibody (DNA topoisomerase I) | 18/32 | 56 |
Anticentromere antibody | 3/31 | 10 |
*Number of patients in whom data were available to the EBMT/EULAR registry. | ||
†Assessed in patients with diffuse SSc only. ‡FVC = forced vital capacity; Tlco = carbon monoxide transfer factor. | ||
Table 1(C) Numbers in the cohort with organ involvement (as described by the contributing doctor) in those patients for whom the data were available | ||
Organ involvement | No/No evaluable patients | % |
Lung | 28/37 | 76 |
Pulmonary hypertension | 7/37 | 19 |
Arterial hypertension | 3/37 | 8 |
Raynaud's phenomenon | 37/40 | 93 |
Renal | 5/37 | 14 |
Oesophageal | 21/36 | 58 |
Gastrointestinal | 4/37 | 11 |