Primary causes of death in 234 patients with SSc
| N | % | |
|---|---|---|
| All death cases | 234 | 100 |
| SSc-related death cases | 128 | 55 |
| Pulmonary | 78 | 33 |
| Pulmonary fibrosis | 45 | 19 |
| Isolated PAH | 33 | 14 |
| Myocardial | 33 | 14 |
| Arrhythmia | 14 | 6 |
| Left heart failure | 8 | 3 |
| Right heart failure | 5 | 2 |
| Biventricular heart failure | 4 | 2 |
| Pericarditis (constriction and/or tamponade) | 2 | 1 |
| Renal | 10 | 4 |
| Renal crisis | 10 | 4 |
| Gastrointestinal | 7 | 3 |
| Non-SSc-related death cases | 96 | 41 |
| Infections | 31 | 13 |
| Pneumonia | 17 | 7 |
| Septicaemia | 14 | 6 |
| Malignancy | 30 | 13 |
| Non-small cell lung cancer | 11 | 5 |
| Small cell lung cancer | 3 | 1 |
| Breast cancer | 4 | 2 |
| Colorectal cancer | 2 | 1 |
| Hepatocellular carcinoma | 2 | 1 |
| Acute myeloid leukaemia | 1 | <1 |
| Lymphoma | 1 | <1 |
| Renal cell carcinoma | 1 | <1 |
| Multiple myeloma | 1 | <1 |
| Pancreas carcinoma | 1 | <1 |
| Angiosarcoma | 1 | <1 |
| Neuroendocrine cancer | 1 | <1 |
| Oesophageal cancer | 1 | <1 |
| Cardiovascular | 28 | 12 |
| Myocardial infarction | 11 | 5 |
| Left heart failure (non-ischaemic) | 6 | 3 |
| Stroke | 6 | 3 |
| Pulmonary embolism | 4 | 2 |
| Arrhythmia (not SSc-related) | 1 | <1 |
| Other | 7 | 3 |
| Gastrointestinal haemorrhage | 3 | 1 |
| Perforated diverticulitis | 1 | <1 |
| Accident | 1 | <1 |
| Iatrogenic | 1 | <1 |
| Suicide | 1 | <1 |
| Cause of death unknown | 10 | 4 |
PAH, pulmonary arterial hypertension; SSc, systemic sclerosis.