PT  - JOURNAL ARTICLE
AU  - S.R. Johnson
AU  - J.T. Granton
TI  - Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus
AID  - 10.1183/09059180.00003811
DP  - 2011 Dec 01
TA  - European Respiratory Review
PG  - 277--286
VI  - 20
IP  - 122
4099  - http://err.ersjournals.com/content/20/122/277.short
4100  - http://err.ersjournals.com/content/20/122/277.full
SO  - EUROPEAN RESPIRATORY REVIEW2011 Dec 01; 20
AB  - 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.