Abstract
Pulmonary arterial hypertension (PAH) is a severe manifestation of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). Due to improvements in the understanding of the pathogenesis of these diseases, improved methodological rigour in the conduct of epidemiological studies and the advent of successful therapies, our understanding of SSc-PAH and SLE-PAH has evolved considerably. In this review we will review the current evidence regarding the prevalence, prognostic factors and survival estimates for SSc-PAH and SLE-PAH. In doing so, we will compare and contrast these two diseases, highlight clinically useful features, discuss methodological limitations of existing data, and draw attention to areas where research is needed.
Footnotes
Provenance
Submitted article, peer reviewed.
Support Statement
S.R. Johnson is supported by a Canadian Institutes of Health Research Clinician Scientist Award and the Norton-Evans Fund for Scleroderma Research.
Statement of Interest
J.T. Granton has received funding to support research from Pfizer and Actelion. He has also acted as an expert witness for Pfizer and consultant for Actelion, Pfizer, Lilly and GlaxoSmithKline. He has received honoraria for speaking from Lilly, Pfizer and Actelion.
- Received April 19, 2011.
- Accepted May 2, 2011.
- ©ERS 2011