Figure 1.

A-C, Increased NE and relative elafin deficiency across PAH subtypes. A, Box-and-whisker plots showing log-transformed NE and elafin levels in patients with PAH (overall and each subtype) compared to those in healthy control participants. Boxplots denote the median, interquartile range, and 1.5 × IQR. P values reflect comparisons of the PAH population overall vs healthy control participants (top of left panel), patients with each PAH subtype vs control participants (top of right panel), and across subtypes (bottom of right panel). B, Graphs showing the distributions of NE and elafin in patients with PAH and healthy control participants, displayed as probability density functions (smoothed histograms resulting from Gaussian kernel estimation). Rug plot hash marks above and below the probability density curves represent patient-level measures. C, Receiver operating characteristic curves displaying the PAH discriminatory power of NE and elafin. Calculated c-statistics (AUC) and ideal discrimination cutoffs (derived from Youden’s index) are indicated. AUC = area under the receiver operating characteristic curve; CHD = congenital heart disease; CTD = connective tissue disease; D&T = drugs and toxins; IPAH = idiopathic pulmonary arterial hypertension; NE = neutrophil elastase; PAH = pulmonary arterial hypertension; PoPH = portopulmonary hypertension.