The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis

Pulmonary arterial hypertension (PAH) is a common complication of scleroderma (SSc) and is a leading cause of death in this population. Advances in our understanding of the pathobiology of PAH have led to the development of therapies that target pathways in the putative pathogenesis of the disease. In addition to early detection strategies to identify patients with PAH, these therapies have led to improvements in morbidity for patients with SSc-associated PAH (PAH-SSc). However, in general, these improvements have been less robust in PAH-SSc than in other forms of PAH. These differences in response to therapy may reflect unique effects of SSc on the right ventricle, whose function is critical to long-term outcomes in PAH. Newer approaches that focus on combination of therapies as initial intervention have led to improvements in time to clinical worsening in PAH-SSc that approach that seen in other forms of PAH. Despite these improvements, lung transplantation should be considered in patients with advanced disease as long-term outcomes for PAH-SSc patients are similar to other forms of PAH.