Demographic and clinical characteristics of the four recurrence categories
| No-DU (n=484) | Episodic (n=137) | Recurrent (n=674) | Chronic (n=164) | Total (N=1459) | |
|---|---|---|---|---|---|
| Gender, n | 484 | 137 | 674 | 164 | 1459 |
| Female, % | 80.6 | 81.8 | 83.7 | 88.4 | 83.0 |
| Age at enrolment, n | 484 | 137 | 674 | 164 | 1459 |
| Mean (SD), years | 55.9 (13.2) | 54.7 (13.7) | 53.2 (14.5) | 50.9 (12.4) | 54.0 (13.8) |
| Age at first RP, n | 418 | 121 | 607 | 148 | 1294 |
| Mean (SD), years | 43.0 (15.3) | 42.9 (15.1) | 39.5 (15.6) | 35.2 (13.6) | 40.4 (15.4) |
| Age at first DU, n | 345 | 111 | 555 | 135 | 1146 |
| Mean (SD), years | 49.5 (14.9) | 48.7 (14.5) | 46.1 (15.2) | 41.7 (14.0) | 46.8 (15.1) |
| SSc classification, n | 482 | 136 | 667 | 163 | 1448 |
| Diffuse SSc, % | 29.7 | 30.1 | 39.3 | 46.6 | 36.0 |
| Limited SSc, % | 56.2 | 58.8 | 51.3 | 44.2 | 52.8 |
| Overlap/mixed CTD, % | 8.9 | 5.1 | 6.3 | 7.4 | 7.2 |
| Other, % | 5.2 | 5.8 | 3.1 | 1.8 | 3.9 |
| Organ manifestations, n | 484 | 137 | 674 | 164 | 1459 |
| GI, % | 54.8 | 52.6 | 58.2 | 63.4 | 57.1 |
| Heart, % | 9.7 | 7.3 | 8.6 | 9.8 | 9.0 |
| Kidney, % | 6.4 | 2.2 | 3.7 | 3.7 | 4.5 |
| Lung fibrosis, % | 34.9 | 38.7 | 39.8 | 52.4 | 39.5 |
| Antibodies, n1/n2 (%) | |||||
| ACA | 165/339 (48.7) | 41/98 (41.8) | 213/528 (40.3) | 34/123 (27.6) | 453/1088 (41.6) |
| ANA | 411/438 (93.8) | 115/124 (92.7) | 592/ 623 (95.0) | 154/158 (97.5) | 1272/1343 (94.7) |
| Anti-Scl 70 | 126/350 (36.0) | 53/101 (52.5) | 263/567 (46.4) | 90/149 (60.4) | 532/1167 (45.6) |
| Anti-U1 RNP | 31/239 (13.0) | 3/65 (4.6) | 32/395 (8.1) | 12/99 (12.1) | 78/798 (9.8) |
| Anti-U3 RNP | 8/150 (5.3) | 0/44 (0.0) | 8/254 (3.1) | 9/68 (13.2) | 25/516 (4.8) |
| RNA polym III | 29/178 (16.3) | 3/44 (6.8) | 27/276 (9.8) | 6/64 (9.4) | 65/562 (11.6) |
| History of previous DU-associated complications/interventions, n1/n2 (%) | |||||
| Critical digital ischaemia | 128/261 (49.0) | 36/91 (39.6) | 167/380 (43.9) | 48/96 (50.0) | 379/828 (45.8) |
| Gangrene | 88/444 (19.8) | 16/126 (12.7) | 157/627 (25.0) | 45/155 (29.0) | 306/1352 (22.6) |
| Autoamputation | 15/448 (3.3) | 7/127 (5.5) | 51/629 (8.1) | 18/156 (11.5) | 91/1360 (6.7) |
| Soft tissue infection requiring systemic antibiotics | 78/420 (18.6) | 39/122 (32.0) | 209/600 (34.8) | 86/149 (57.7) | 412/1291 (31.9) |
| Osteomyelitis | 15/438 (3.4) | 3/124 (2.4) | 22/628 (3.5) | 12/153 (7.8) | 52/1343 (3.9) |
| Hospitalisation for DUs | 164/444 (36.9) | 54/128 (42.2) | 298/633 (47.1) | 93/155 (60.0) | 609/1360 (44.8) |
| Upper limb sympathectomy | 17/442 (3.8) | 6/125 (4.8) | 21/621 (3.4) | 14/150 (9.3) | 58/1338 (4.3) |
| Digital sympathectomy | 8/441 (1.8) | 0/125 (0.0) | 14/619 (2.3) | 6/148 (4.1) | 28/1333 (2.1) |
| Arterial reconstruction | 5/442 (1.1) | 1/125 (0.8) | 3/617 (0.5) | 2/149 (1.3) | 11/1333 (0.8) |
| Arthrodesis | 5/388 (1.3) | 3/106 (2.8) | 12/539 (2.2) | 6/124 (4.8) | 26/1157 (2.2) |
| Debridement | 22/384 (5.7) | 6/106 (5.7) | 68/537(12.7) | 27/125 (21.6) | 123/1152 (10.7) |
| Surgical amputation | 23/390 (5.9) | 7/106 (6.6) | 54/542 (10.0) | 20/126 (15.9) | 104/1164 (8.9) |
| Use of parenteral prostanoids | 223/439 (50.8) | 70/127 (55.1) | 394/608 (64.8) | 113/150 (75.3) | 800/1324 (60.4) |
| Number of DUs at enrolment, n | 481 | 135 | 668 | 161 | 1445 |
| 0*, % | 66.1 | 48.9 | 31.7 | 10.6 | 42.4 |
| 1–2, % | 24.9 | 37.0 | 45.2 | 36.0 | 36.7 |
| 3+, % | 8.9 | 14.1 | 23.1 | 53.4 | 20.9 |
| Ongoing medication at enrolment, n | 484 | 137 | 674 | 164 | 1459 |
| Analgesics and anti-inflammatories, % | 52.7 | 60.6 | 58.9 | 67.7 | 58.0 |
| Immunosuppressants, % | 37.0 | 34.3 | 31.8 | 34.8 | 34.1 |
| Systemic antibiotics, % | 6.0 | 13.9 | 18.8 | 27.4 | 15.1 |
| ERA, any combination, % | 41.5 | 38.7 | 49.6 | 46.3 | 45.5 |
| Prostacyclins, % | 27.1 | 28.5 | 42.1 | 43.3 | 36.0 |
| CCB, % | 43.6 | 41.6 | 47.5 | 53.7 | 46.3 |
| PDE5i, % | 4.8 | 4.4 | 6.1 | 4.9 | 5.3 |
| ERA+PDE5i, % | 2.1 | 2.2 | 1.5 | 1.2 | 1.7 |
| ERA+prostacyclin, % | 10.3 | 10.9 | 17.7 | 19.5 | 14.8 |
| PDE5i+prostacyclin, % | 0.6 | 0.0 | 2.5 | 2.4 | 1.6 |
| Other medications, % | 74.8 | 73.7 | 71.7 | 63.4 | 72.0 |
| Topical treatment of DUs at enrolment, n | 484 | 137 | 674 | 164 | 1459 |
| Any, % | 7.6 | 15.3 | 24.8 | 34.8 | 19.3 |
| Topical antibiotics, % | 1.9 | 3.6 | 6.5 | 10.4 | 5.1 |
| Proteolytic enzymes, % | 0.8 | 0.0 | 1.2 | 3.0 | 1.2 |
| Alginates, % | 0.2 | 0.7 | 1.3 | 1.8 | 1.0 |
| Growth factors, % | 0.4 | 0.0 | 0.7 | 1.8 | 0.7 |
| Dry dressing, % | 4.1 | 6.6 | 10.8 | 13.4 | 8.5 |
| Non-adhesive dressing, % | 1.0 | 3.6 | 7.1 | 14.6 | 5.6 |
| Hydro-colloids, % | 2.3 | 5.1 | 7.4 | 12.8 | 6.1 |
| Bioengineered skin, % | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 |
*All patients had prior DUs.
ACA, anticentromere antibodies; ANA, antinuclear antibodies; CCB, calcium channel blocker; CTD, connective tissue disease; DUs, digital ulcers; ERA, endothelin receptor antagonist; GI, gastrointestinal; n, n1, number of patients; n2, total number of patients for whom information was available; PDE5i, phosphodiesterase 5 inhibitor; RP, Raynaud's phenomenon; SSc, systemic sclerosis.