Frequency of organ complications in systemic sclerosis (SSc) from the Canadian Scleroderma Research Group (CSRG).
| Baseline Characteristic | All Patients | Limited SSc | Diffuse SSc | Early dcSSc (≤ 3 yrs) | Late dcSSc (> 3 yrs) |
|---|---|---|---|---|---|
| No. (% from total group) | 1043 | 647 (62) | 396 (38) | 117 (10.6) | 269 (25.6) |
| Age, yrs (SD) | 55.39 (12.13) | 57.06 (11.77) | 52.64 (12.31) | 51.2 (13.21) | 53.34 (11.77) |
| Female, n (%) | 986 (86.1) | 575 (88.9) | 319 (80.6) | 90 (76.9) | 221 (82.2) |
| Disease duration, yr (SD) | 10.99 (9.50) | 12.37 (10.06) | 9.02 (8.45) | 1.46 (0.74) | 12.30 (8.16) |
| Scleroderma renal crisis, % | 4.3 | 1.9 | 8.4 | 8.5 | 8.2 |
| Cardiomyopathy (%) | 9/496 (1.8) | 5/273 (1.8) | 4/190 (2.1) | 1/59 (1.7) | 3/129 (2.3) |
| Arrhythmias (%) | 70/280 (25.0) | 38/160 (23.8) | 26/100 (26.0) | 7/24 (29.2) | 18/74 (24.3) |
| Moderate to large pericardial effusion (%) | 16/949 (1.7) | 10/543 (1.8) | 4/332 (1.2) | 3/98 (3.1) | 1/225 (0.4) |
| Systolic pulmonary artery pressure ≥ 45 mm Hg (%) | 123/668 (18.4) | 67/385 (17.4) | 48/228 (21.1) | 13/73 (17.8) | 34/149 (22.8) |
| Forced vital capacity < 70% predicted (%) | 125/946 (13.2) | 56/539 (10.4) | 60/338 (17.8) | 15/104 (14.4) | 42/228 (18.4) |
| sPAP ≥ 45 mm Hg & FVC < 70% (%) | 35/952 (3.7) | 13/549 (2.4) | 21/331 (6.3) | 6/101 (5.9) | 14/224 (6.3) |
| Hyperalimentation (%) | 31/1121 (2.8) | 9/642 (1.4) | 20/392 (5.1) | 3/117 (2.6) | 16/269 (5.9) |
| Intestinal pseudoobstruction (%) | 44/1120 (3.9) | 24/641 (3.7) | 18/392 (4.6) | 0/117 (2.6) | 17/269 (6.3) |
| Proximal limb girdle weakness and/or neck weakness (%) | 181/1114 (16.2) | 78/639 (12.2) | 93/389 (23.9) | 33/115 (28.7) | 57/266 (21.4) |
| Arthritis (SJC ≥ 1 and TJC ≥ 1), % | 10.5 | 11.1 | 10.2 | 12.1 | 9.8 |
| Active digital ulcer (%) | 93/1121 (8.3) | 50/644 (7.8) | 40/393 (10.2) | 11/117 (9.4) | 28/267 (10.5) |
| Digital necrosis/gangrene (%) | 12/1123 (1.1) | 9/644 (1.4) | 3/395 (0.8) | 1/117 (0.9) | 2/269 (0.7) |
| Digital loss/amputation (%) | 66/1124 (5.9) | 39/645 (6.0) | 27/395 (6.8) | 2/117 (1.7) | 24/269 (8.9) |
| Modified Rodnan skin score (SD) | 10.04 (9.54) | 5.94 (5.07) | 18.34 (10.29) | 22.20 (10.14) | 16.59 (9.85) |
| Modified Rodnan skin score > 20 (%) | 155/1102 (14.1) | 13/632 (2.1) | 142/387 (36.7) | 63/116 (54.3) | 75/264 (28.4) |
dcSSc: diffuse cutaneous systemic sclerosis; lcSSc: limited cutaneous systemic sclerosis; sPAP: systolic pulmonary artery pressure; FVC: forced vital capacity; SJC: swollen joint count; TJC: tender joint count.