title: Recent advances in scleroderma-associated pulmonary hypertension.
abstract: OBJECTIVE
  This review summarizes recent advances in pulmonary hypertension, a leading cause of morbidity and mortality in scleroderma (SSc).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Although there have been many recent advances in SSc-pulmonary hypertension, further research is needed in order to prevent/cure this deadly complication.
authors.affiliation: Respiratory Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
authors.forename: Kristin B
authors.initials: KB
authors.lastname: Highland
mesh_terms: D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007249:Inflammation / Q000150:complications; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*