The management of pulmonary arterial hypertension in the setting of systemic sclerosis

Pulmonary hypertension (PH) commonly complicates systemic sclerosis (SSc). PH related to SSc can develop in multiple ways and thus be classified in any of the five groups of pulmonary hypertension as defined by the World Health Organization. PH significantly impacts morbidity and mortality in SSc and, along with interstitial lung disease, is currently the leading cause of death in this population. While advances in therapeutics have led to improvements in symptoms, functional capacity, and outcomes in patients with pulmonary arterial hypertension (PAH), the response to therapy in PAH related to SSc (SSc-PAH) is, in general, less robust than other forms of PAH. Further, there are few randomized clinical trials of these therapies in other forms of PH, and thus, treatment in these populations is challenging. Newer therapeutic strategies, such as simultaneously targeting multiple pathways involved in the putative pathogenesis of the disease or utilizing inhaled therapies, may offer improved outcomes for SSc-PAH and lead to randomized trials in other forms of SSc-related PH. Focusing on supportive therapy, such as pulmonary rehabilitation, may also be important in the management of this complex disease.