Enrolment characteristics and patient demographics according to gangrene status*
| Never† gangrene (n=3787) | Ever‡ gangrene (n=855) | Current gangrene (n=258)§ | |
|---|---|---|---|
| Gender | |||
| Female, % | 82.1 | 77.7 | 77.5 |
| Age at enrolment | |||
| Mean (95% CI), years | 54.4 (53.9 to 54.8) | 54.8 (53.9 to 55.8) | 52.8 (50.9 to 54.7) |
| Smoking status | |||
| n | 3386 | 757 | 233 |
| Current, % | 14.4 | 17.6 | 24.0 |
| Former, % | 23.3 | 25.6 | 17.6 |
| Never, % | 62.3 | 56.8 | 58.4 |
| Pack-years of smoking | |||
| n | 868 | 206 | 73 |
| Mean (95% CI) | 37.8 (31.3 to 44.3) | 37.9 (27.5 to 48.4) | 44.9 (24.9 to 64.9) |
| Age at first RP | |||
| n | 3409 | 752 | 229 |
| Mean (95% CI), years | 41.3 (40.8 to 41.8) | 40.7 (39.6 to 41.8) | 41.2 (39.0 to 43.3) |
| Age at first DU | |||
| n | 3000 | 700 | 218 |
| Mean (95% CI), years | 47.6 (47.1 to 48.2) | 47.1 (45.9 to 48.2) | 48.3 (46.1 to 50.5) |
| SSc cutaneous subset | |||
| n | 3774 | 850 | 256 |
| Diffuse SSc, % | 37.7 | 32.0 | 33.6 |
| Limited SSc, % | 52.3 | 58.2 | 54.3 |
| Overlap, % | 6.5 | 6.0 | 7.8 |
| Other, % | 3.6 | 3.8 | 4.3 |
| Organ manifestations | |||
| n | 3787 | 855 | 258 |
| GI tract, % | 54.0 | 56.8 | 46.5 |
| Lung fibrosis, % | 40.4 | 40.1 | 38.0 |
| PAH, % | 12.1 | 15.2 | 13.2 |
| Heart, % | 9.9 | 10.9 | 12.4 |
| Kidney, % | 4.1 | 6.0 | 5.8 |
| Time from first RP to enrolment visit | |||
| n | 3409 | 752 | 229 |
| Mean (95% CI), years | 13.1 (12.8 to 13.5) | 14.4 (13.6 to 15.3) | 11.9 (10.4 to 13.5) |
| Time from first DU to enrolment visit | |||
| n | 3000 | 700 | 218 |
| Mean (95% CI), years | 5.9 (5.7 to 6.2) | 7.4 (6.8 to 8.0) | 4.6 (3.8 to 5.5) |
| Antibodies, n1/n2 (%) | |||
| ACA | 1184/2942 (40.2) | 303/668 (45.4) | 88/216 (40.7) |
| ANA | 3307/3511 (94.2) | 750/785 (95.5) | 226/238 (95.0) |
| Anti-Scl 70 | 1397/3145 (44.4) | 282/690 (40.9) | 87/218 (39.9) |
| Anti-U1 RNP | 170/2158 (7.9) | 52/470 (11.1) | 17/151 (11.3) |
| Anti-U3 RNP | 59/1534 (3.8) | 19/300 (6.3) | 4/104 (3.8) |
| RNA polymerase III | 127/1584 (8.0) | 25/323 (7.7) | 6/103 (5.8) |
| Employed/self-employed, n (%) | 983/2674 (36.8) | 167/564 (29.6) | 75/207 (36.2) |
| History of complications/interventions, % (95% CI)¶ | |||
| Critical digital ischaemia | 30.1 (28.5 to 31.8) | 82.2 (78.6 to 85.4) | 69.4 (61.6 to 76.4) |
| Gangrene | – | 91.7 (89.7 to 93.5) | 71.9 (65.9 to 77.4) |
| Autoamputation | 3.1 (2.6 to 3.7) | 24.1 (21.2 to 27.2) | 15.9 (11.6 to 21.1) |
| Soft-tissue infection requiring systemic antibiotics | 23.9 (22.5 to 25.3) | 53.5 (49.9 to 57.0) | 44.5 (38.1 to 51.1) |
| Osteomyelitis | 1.3 (0.9 to 1.7) | 11.9 (9.7 to 14.3) | 7.4 (4.4 to 11.4) |
| Hospitalisations for DUs | 32.7 (31.2 to 34.2) | 70.1 (66.9 to 73.2) | 58.9 (52.5 to 65.2) |
| Upper limb sympathectomy | 2.2 (1.8 to 2.7) | 8.8 (6.9 to 10.9) | 7.2 (4.2 to 11.2) |
| Digital sympathectomy | 1.4 (1.0 to 1.8) | 4.8 (3.4 to 6.5) | 3.4 (1.5 to 6.6) |
| Arterial reconstruction | 0.7 (0.5 to 1.0) | 2.1 (1.3 to 3.4) | 4.3 (2.1 to 7.7) |
| Arthrodesis | 1.4 (1.0 to 1.9) | 5.7 (4.1 to 7.6) | 2.0 (0.5 to 4.9) |
| Debridement | 7.5 (6.6 to 8.4) | 25.7 (22.5 to 29.1) | 21.0 (15.6 to 27.2) |
| Surgical amputation | 2.4 (1.9 to 3.0) | 34.0 (30.5 to 37.5) | 18.9 (13.8 to 24.8) |
| Use of parenteral prostanoids | 51.6 (49.9 to 53.2) | 74.4 (71.2 to 77.4) | 74.4 (68.3 to 79.8) |
| Prior DUs, n1/n2 (%) | 3759/3787 (99.3) | 852/855 (99.6) | 255/258 (98.8) |
| Ongoing medications, % | |||
| n | 3787 | 855 | 258 |
| Analgesics and anti-inflammatories | 52.4 | 60.6 | 65.1 |
| Immunosuppressants | 33.5 | 28.2 | 29.5 |
| Systemic antibiotics | 13.3 | 19.6 | 36.0 |
| ERAs | 39.9 | 52.0 | 50.4 |
| CCBs | 46.0 | 52.5 | 53.1 |
| Prostacyclins | 35.0 | 36.5 | 51.9 |
| PDE-5i | 5.9 | 7.6 | 5.8 |
| Topical DU treatments | 19.1 | 24.4 | 36.8 |
| Other medications | 64.8 | 74.2 | 67.1 |
| ERA+PDE-5i | 2.2 | 3.3 | 2.7 |
| ERA+prostacyclin | 14.3 | 18.5 | 24.4 |
| PDE-5i+prostacyclin | 1.7 | 2.8 | 3.1 |
| ERA+PDE-5i+prostacyclin | 0.8 | 1.5 | 1.6 |
| ERA only** | 24.1 | 31.8 | 24.8 |
*Only patients who provided information on gangrene status (n=4642/4944) were categorised.
†Patients with no past and no current gangrene.
‡Patients with past and/or current gangrene.
§Patients with current gangrene at enrolment. The current gangrene group is a subset of the ‘ever gangrene’ group.
¶Data include only patients who provided information on the given item.
**Out of ERA, PDE-5i and prostacyclins, only ERA is ticked.
ACA, anticentromere antibody; ANA, antinuclear antibody; CCB, calcium channel blocker; DU, digital ulcer; ERA, endothelin receptor antagonist; GI, gastrointestinal; n1/n2, n patients tested positive/n patients who had the test done; PAH, pulmonary arterial hypertension; PDE-5i, phosphodiesterase-type 5 inhibitor; RNP, ribonucleic protein; RP, Raynaud's phenomenon; SSc, systemic sclerosis.