Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a Swedish single centre: prognosis still poor and prediction difficult

摘要: follow-up. Results: The 1-, 2-, 3-, and 4-year survival rates were 86, 59, 39, 22%, respectively, from diagnosis of PAH. hazard ratio for total mortality in the SSc-PAH group was 3.2 [95% confidence interval (CI) 1.8–5.7] compared to SSc without PAH (p < 0.001). Risk factors at baseline development were: limited skin involvement, low diffusing capacity lung carbon monoxide (DL CO ), high N-terminal pro-brain natriuretic peptide (NTProBNP), increased estimated systolic pulmonary arterial pressure (ESPAP), presence teleangiectases. Severe peripheral vascular disease requiring iloprost treatment during follow-up associated with an eightfold risk Conclusion: Despite modern yearly screening by echocardiography, is still our cohort. identified should be assessed select patients eligible right heart catheterization (RHC) make earlier diagnosis.