Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis

Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis SSc-pulmonary disease is a devastating condition Pulmonary disease is a frequent complication of systemic sclerosis (SSc) and is at the same time challenging for the treating physician. Besides lung fibrosis, pulmonary arterial hypertension (PAH)-defined as a mean pulmonary artery pressure above 25 mmHg at rest or above 30 mmHg during exercise by right heart catheterization-can occur both in the limited and diffuse subset of SSc and leads to right cardiac overload with exercise intolerance, dyspnea and arrhythmia The natural history of PAH associated with SSc (APAH/SSc) is devastating. While 1-, 3and 5-yr survival rates after diagnosis in the idiopathic form (IPAH) are 68, 48 and 34% (median life expectancy 2.8 yrs) in untreated patients, several studies have shown that the prognosis in APAH/SSc is even worse. Median survival in untreated patients is only 12 months, and the risk of death is nearly tripled