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 |