Management of the Scleroderma Patient with Pulmonary Arterial Hypertension Failing Initial Therapy

Pulmonary arterial hypertension commonly develops in patients with scleroderma (PAH-SSc) and portends poor survival. Advances in therapeutics have led to improvements in disease morbidity for patients, but mortality remains unacceptably high. Consensus guidelines recommend application of risk assessment to determine treatment strategy. In this chapter, a case presentation will be used to highlight the use of risk assessment in the management of a patient with PAH-SSc at the time of diagnosis through disease progression. Development of comorbidities and complications, both common and unusual, and the role of lung transplantation and palliative care in the management of these complex patients will be presented.