Pericardial Abnormalities Predict the Presence of Echocardiographically Defined Pulmonary Arterial Hypertension in Systemic Sclerosis-Related Interstitial Lung Disease

摘要

Objectives: To determine the prevalence and significance of pericardial abnormalities in systemicnsclerosis (SSc)-related interstitial lung disease (ILD).nMethods: Retrospective study of 41 subjects with SSc-related ILD who underwent evaluationnincluding thoracic high-resolution CT (HRCT) imaging, transthoracic echocardiography (TTE),nand pulmonary function testing. HRCT review evaluated the pericardium for the presence ofnpericardial effusion (PEf), thickness of the anterior pericardial recess (APR) [abnormal defined asn> 10 mm], and pericardial thickening as calculated by total pericardial score (TPS) [abnormalndefined as > 8 mm]. Pulmonary arterial hypertension (PAH) was defined as a pulmonary arterynpressure > 35 mm Hg estimated by TTE.nResults: Fifty-nine percent had an abnormal pericardium, 49% had a PEf, 56% had an abnormalnAPR, and 49% had an abnormal TPS. An abnormal pericardium was more common in men thannwomen. Subjects with and without pericardial abnormalities were otherwise similar with respectnto age, SSc classification, autoantibodies, ILD radiographic pattern, and presence of esophagealndilation. Both groups had similar median percentage of predicted total lung capacity, percentagenof predicted FVC, percentage of predicted FEV1, and percentage of predicted diffusion capacitynof the lung for carbon monoxide. Subjects with pericardial abnormalities were more likely to havencoexistent PAH (35% vs 75%; p u0001 0.02) and a higher median right ventricular systolic pressuren(31 mm Hg vs 44 mm Hg; p u0001 0.03). Multiple logistic regression revealed that TPS was the bestnindividual predictor of the presence of TTE-defined PAH.nConclusions: In patients with SSc-related ILD, pericardial abnormalities are commonly seen onnHRCT, and their presence is strongly associated with echocardiographically defined PAH, withnabnormal TPS as the best individual predictor.