General demographic features of 405 patients with systemic sclerosis (SSc). Values are median ± SE of the mean. Nine patients who were lost to followup were excluded from survival or standardized mortality ratio analyses.
| Characteristic | All Patients | Surviving Patients, n (%) | Dead Patients, n (%) | p |
|---|---|---|---|---|
| No. patients | 405 | 310 (76.5) | 86 (21.2) | — |
| Men, n (%) | 29 (7.2) | 19 (6.1) | 9 (10.5) | 0.1616 |
| dcSSc, n (%) | 132 (32.6) | 86 (27.7) | 46 (53.5) | < 0.0001 |
| Age at onset, yrs (range) | 47 ± 0.7 (5–78) | 47 ± 1.6 (5–78) | 47 ± 0.8 (9–73) | 0.9029 |
| Onset to diagnosis, yrs (range) | 2 ± 0.4 (0–44) | 2 ± 0.5 (0–44) | 3 ± 0.7 (0–35) | 0.6290 |
| Disease duration, yrs (range) | 14 ± 0.6 (1–59) | 15 ± 0.7 (1–59) | 11 ± 1.2 (1–57) | 0.0230 |
| Organ involvement | ||||
| GI tract | 187 | 137 (44.2) | 49 (57.0) | 0.0356 |
| Lung | 204 | 125 (40.3) | 75 (87.2) | < 0.0001 |
| Heart | 79 | 35 (11.3) | 43 (50.0) | < 0.0001 |
| Kidney | 60 | 34 (11.0) | 25 (29.1) | 0.0001 |
| Scleroderma renal crisis | 13 | 6 (1.9) | 7 (8.1) | 0.0100 |
| Pulmonary arterial hypertension | 65 | 36 (11.6) | 27 (31.4) | < 0.0001 |
| Overlap of connective tissue disease | ||||
| Systemic lupus erythematosus | 21 | 18 (5.8) | 3 (3.5) | 0.5870 |
| Rheumatoid arthritis | 19 | 15 (4.8) | 4 (4.7) | 1.0000 |
| Sjögren’s syndrome | 51 | 48 (15.5) | 3 (3.5) | 0.0018 |
| Myositis | 22 | 13 (4.2) | 8 (9.3) | 0.0970 |
DcSSc: diffuse cutaneous systemic sclerosis; GI: gastrointestinal.