Continuous variables are presented as median and interquartile range while categorical variables are presented as ratio. lSSc - localized systemic sclerosis. dcSSc - diffuse systemic sclerosis. ILD - interstitial lung disease. %DLCO – percent diffusing capacity for carbon monoxide. PHT - pulmonary hypertension. PAPs – pulmonary arterial pressure. FVC - forced vital capacity. CRP – C-reactive protein. ESR - erythrocyte sedimentation rate. ACE - angiotensin convertase enzyme. N/A- not applicable. This evidence concerns the gene ACE and pulmonary hypertension, primary, 1.