Recent advances in scleroderma-associated pulmonary hypertension

This review summarizes recent advances in pulmonary hypertension, a leading cause of morbidity and mortality in scleroderma (SSc).

Although WHO Group I pulmonary arterial hypertension (PAH) is the most common cause of pulmonary hypertension, all WHO Groups can occur. PAH is now a criterion for the diagnosis of SSc. Results of recent research have resulted in greater insight into the epidemiology of SSc-pulmonary hypertension with regard to prevalence, incidence and clinical risk factors. There is also greater understanding of the role of inflammation in the pathogenesis of SSc-PAH. Advances have also been made in the evaluation and screening of patients with SSc-PAH, and early detection has been shown to improve survival in a disease that typically has worse outcomes than other forms of PAH. Finally, recommendations have been made with regard to goal-directed therapy.

Although there have been many recent advances in SSc-pulmonary hypertension, further research is needed in order to prevent/cure this deadly complication.