Table 1

Primary causes of death in 234 patients with SSc

All death cases234100
SSc-related death cases12855
  Pulmonary fibrosis4519
  Isolated PAH3314
  Left heart failure83
  Right heart failure52
  Biventricular heart failure42
  Pericarditis (constriction and/or tamponade)21
  Renal crisis104
Non-SSc-related death cases9641
  Non-small cell lung cancer115
  Small cell lung cancer31
  Breast cancer42
  Colorectal cancer21
  Hepatocellular carcinoma21
  Acute myeloid leukaemia1<1
  Renal cell carcinoma1<1
  Multiple myeloma1<1
  Pancreas carcinoma1<1
  Neuroendocrine cancer1<1
  Oesophageal cancer1<1
  Myocardial infarction115
  Left heart failure (non-ischaemic)63
  Pulmonary embolism42
  Arrhythmia (not SSc-related)1<1
  Gastrointestinal haemorrhage31
  Perforated diverticulitis1<1
Cause of death unknown104
  • PAH, pulmonary arterial hypertension; SSc, systemic sclerosis.