Johnson SR et al. - HLA-DRw52 and -DRw6, initial sPAP > 60 mmHg, mRAP, and shorter time between systemic sclerosis (SSc) onset and observed pulmonary arterial hypertension (PAH) were associated with decreased survival; however, methodologic quality of study reporting was variable. Methods
Aim was to (i) identify factors associated with survival in SSc-PAH and (ii) to evaluate the methodologic quality of prognostic studies against current standards
A systematic review to identify studies evaluating factors associated with survival in SSc-PAH
The methodologic quality of each study was evaluated using a methodologic quality index
Results
HLA-DRw6, HLA-DRw52, initial systolic pulmonary artery pressure (sPAP) >60 mmHg, elevated mean right atrial pressure (mRAP), and shorter time between SSc onset and observed PAH were associated with decreased survival
Age >50 yrs, male sex, limited subtypes, pulmonary fibrosis, change in pulmonary vascular resistance, anti-centromere and anti-ScL-70 abs were not definitively associated with survival
Attributes of participants, prognostic factors, and outcome measures were well reported
Study attrition, confounding, and analysis were not well reported
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