CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH

Inclusion Criteria: * Patients who have scleroderma ILD will be defined as having a total lung capacity of less than 80% predicted and CT evidence of fibrosis. The degree of fibrosis will be scored by a radiologist using the CT comparative scoring method of Wells et al (13). * Pulmonary Hypertension (PH) as defined as resting mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg with a wedge pressure of ≤ 15 mmHg during right heart catheterization. * Stable ILD as evident by a stable FEV1 and FVC for 3 months prior to the initiation of the study, and be pulmonary arterial hypertension (PAH)-targeted treatment naïve. Exclusion Criteria: * Patients with a left ventricular ejection fraction \<50% or clinical, echocardiographic, and/or catheterization data consistent with heart failure with preserved ejection fraction (HFpEF) and/or moderate-severe aortic or mitral valve abnormality * Patients with severe restrictive lung disease (FVC\<40% predicted) and/or obstructive lung disease (FEV1 \<55% predicted and FEV1/FVC \<70%). * Patients with radiographic combined pulmonary fibrosis/emphysema (CPFE) will also be excluded if imaging shows predominant emphysema and/or obstruction is moderately severe (FEV1\<30%) * Patients with a history of pulmonary embolism within the last three months or evidence of chronic pulmonary embolism. * Patients with a known contraindication to right heart catheterization. * Patients whom have received active or previous pulmonary vasoactive medication with…