Association of FVC/DLCO with pulmonary hypertension risk and interstitial disease in systemic sclerosis patients

2020 
Introduction: Pulmonary hypertension (PH) and interstitial lung disease (ILD) are associated with poor outcomes in patients with systemic sclerosis (SSc). Forced Vital Capacity/Diffusion capacity for carbon monoxide(FVC/DLCO) has been proposed as a mortality predictor marker. Objective: To analyze the association of FVC/DLCO with PH and ILD in a Latin-American cohort. Methods: Retrospective cohort study. Patients who complied ACR/EULAR diagnosis criteria of SSc during 2014 to 2019 were analyzed. 75 offered complete clinical record, high resolution computed tomography (HRCT), Pulmonary function test (PFT) and Echocardiography at baseline. We defined “PH-risk” group as the presence of ≥1 feature of ≥2 echocardiographic categories described in the 6th world symposium (ventricle / pulmonary artery / inferior vena cava-right atrium) or a pulmonary artery systolic pressure (PASP)> 35 mmHg. ILD was defined as an extent of >20% in the HRCT. Results: Among the 75 patients: 72 (96%) were women and mean age was 60 (+/-13) years. When comparing the 2 groups: Conclusion: In our cohort FVC/DLCO was significantly higher between the PH-risk patients, regardless of the presence of ILD. This index could be used as a PH screening tool even in patients with SSc associated ILD.
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