Long-term morbidity and mortality outcomes of SLS I and II participants
| SLS I | SLS II | |||
| CYC (n=79) | Placebo (n=79) | CYC (n=73) | MMF (n=69) | |
| Subject status | ||||
| Alive | 29 (37%) | 29 (37%) | 50 (68%) | 51 (74%) |
| Lost to follow-up* | 10 (13%) | 22 (28%) | 7 (10%) | 4 (6%) |
| No data available | 2 (3%) | 0 | 0 | 0 |
| Deceased | 38 (48%) | 28 (35%) | 16 (22%) | 14 (20%) |
| Death related to SSc | 12 | 12 | 7 | 8 |
| Death unrelated to SSc | 6 | 4 | 8 | 3 |
| Unknown if related to SSc | 20 | 12 | 1 | 3 |
| Cause of death† | ||||
| Respiratory failure (ILD)‡ | 9 | 9 | 6 | 7 |
| Aspiration | 0 | 0 | 2 | 0 |
| Pulmonary hypertension | 0 | 0 | 0 | 1 |
| Myocardial infarction | 0 | 0 | 1 | 0 |
| Cancer | 4 | 3 | 2 | 1 |
| Heart failure | 4 | 6 | 2 | 1 |
| Renal failure | 2 | 0 | 0 | 0 |
| Complications from lung transplantation | 1 | 0 | 0 | 0 |
| Complications from hip fracture | 0 | 0 | 1 | 0 |
| Gastrointestinal tract failure | 1 | 1 | 0 | 0 |
| Sepsis | 1 | 0 | 0 | 0 |
| Seizures | 0 | 1 | 0 | 0 |
| Infection | 0 | 1 | 0 | 0 |
| Other | 1 | 0 | 0 | 0 |
| Unknown | 19 | 11 | 2 | 4 |
| Organ failure | ||||
| No organ failure | 63 (80%) | 60 (76%) | 58 (79%) | 58 (84%) |
| Any organ failure | 14 (18%) | 19 (24%) | 8 (11%) | 7 (10%) |
| No data available | 2 (3%) | 0 | 7 (10%) | 4 (6%) |
| Type of organ failure† | ||||
| Supplementary oxygen use | 12 | 17 | 5 | 6 |
| Lung transplant§ | 1 | 2 | 0 | 1 |
| Dialysis | 2 | 0 | 0 | 1 |
| Total parenteral nutrition | 0 | 0 | 1 | 1 |
| Cardiac ablation | 2 | 1 | 1 | 2 |
| Pacemaker | 0 | 1 | 1 | 0 |
| Malignancy | 7 | 6 | 0 | 1 |
| Development of pulmonary hypertension | Not collected | Not collected | 2 | 9 |
| Median follow-up time (IQR), months | 97.4 (37.3–121.9) | 86.7 (32.3–120.2) | 40.78 (24.9–54.5) | 42.2 (36.4–58.7) |
CYC, cyclophosphamide; ILD, interstitial lung disease; MMF, mycophenolate ; SLS, Scleroderma Lung Studies; SSc, systemic sclerosis.
*These patients were not in death registries, but were unreachable.
†Subjects could have more than one cause of death and type of organ failure recorded. In SLS I CYC group, 1 person died of sepsis and respiratory failure, 1 died of heart and renal failure, 1 died of respiratory failure and renal failure, and 1 died of respiratory failure and gastrointestinal failure. In SLS I placebo group, 1 person died of respiratory failure and heart failure, 1 died of respiratory failure and gastrointestinal failure, 1 died of respiratory failure and infection, and 1 died of respiratory failure and seizures.
‡In the SLS I study, 2 deaths (1 CYC, 1 placebo) due to ‘Respiratory Failure’ were not attributed to underlying SSc based on the case report form.
§No patients received heart, kidney, liver or bone marrow transplantations during the follow-up period.