Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: Impact of interstitial lung disease

Abstract

Survival in SSc complicated by PH remains poor despite currently available treatment options. While therapy may be associated with improved survival in PAH compared with historical controls, the prognosis for patients with ILD-associated PH is particularly grim. Early diagnosis and treatment may improve outcomes since worsening hemodynamic factors were associated with reduced survival.