Pulmonary Hypertension in Scleroderma
Pulmonary Hypertension in Scleroderma
The natural history of SSc–PAH is subclinical progression of vascular injury until dyspnea results. Once PAH is recognized, the median survival during the era before pulmonary vasodilator therapy was available was 1–3 years. The eventual cause of death in SSc–PAH is usually the result of right heart failure or refractory hypoxemia. As such, the combination of interstitial lung disease and SSc–PAH portends a much worse prognosis than isolated SSc–PAH; in effect, two large studies have shown less than 50% survival at 2 years in patients with both entities. The clinical considerations regarding the effect of early screening and treatment in SSc–PAH morbidity and mortality, as well as issues of management are discussed below.
Natural History
The natural history of SSc–PAH is subclinical progression of vascular injury until dyspnea results. Once PAH is recognized, the median survival during the era before pulmonary vasodilator therapy was available was 1–3 years. The eventual cause of death in SSc–PAH is usually the result of right heart failure or refractory hypoxemia. As such, the combination of interstitial lung disease and SSc–PAH portends a much worse prognosis than isolated SSc–PAH; in effect, two large studies have shown less than 50% survival at 2 years in patients with both entities. The clinical considerations regarding the effect of early screening and treatment in SSc–PAH morbidity and mortality, as well as issues of management are discussed below.
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