Chest
Volume 131, Issue 4, April 2007, Pages 988-992
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Original Research
Pulmonary Hypertension
Pericardial Abnormalities Predict the Presence of Echocardiographically Defined Pulmonary Arterial Hypertension in Systemic Sclerosis-Related Interstitial Lung Disease

https://doi.org/10.1378/chest.06-2317Get rights and content

Objectives

To determine the prevalence and significance of pericardial abnormalities in systemic sclerosis (SSc)-related interstitial lung disease (ILD).

Methods

Retrospective study of 41 subjects with SSc-related ILD who underwent evaluation including thoracic high-resolution CT (HRCT) imaging, transthoracic echocardiography (TTE), and pulmonary function testing. HRCT review evaluated the pericardium for the presence of pericardial effusion (PEf), thickness of the anterior pericardial recess (APR) [abnormal defined as > 10 mm], and pericardial thickening as calculated by total pericardial score (TPS) [abnormal defined as > 8 mm]. Pulmonary arterial hypertension (PAH) was defined as a pulmonary artery pressure > 35 mm Hg estimated by TTE.

Results

Fifty-nine percent had an abnormal pericardium, 49% had a PEf, 56% had an abnormal APR, and 49% had an abnormal TPS. An abnormal pericardium was more common in men than women. Subjects with and without pericardial abnormalities were otherwise similar with respect to age, SSc classification, autoantibodies, ILD radiographic pattern, and presence of esophageal dilation. Both groups had similar median percentage of predicted total lung capacity, percentage of predicted FVC, percentage of predicted FEV1, and percentage of predicted diffusion capacity of the lung for carbon monoxide. Subjects with pericardial abnormalities were more likely to have coexistent PAH (35% vs 75%; p = 0.02) and a higher median right ventricular systolic pressure (31 mm Hg vs 44 mm Hg; p = 0.03). Multiple logistic regression revealed that TPS was the best individual predictor of the presence of TTE-defined PAH.

Conclusions

In patients with SSc-related ILD, pericardial abnormalities are commonly seen on HRCT, and their presence is strongly associated with echocardiographically defined PAH, with abnormal TPS as the best individual predictor.

Section snippets

Subjects

We identified 41 subjects who consented to enroll in our institutional review board-approved and Health Insurance Portability and Accountability Act-compliant ILD database with SSc-related ILD who had undergone a comprehensive evaluation that included the following: history and physical examination, rheumatologic serologic testing, pulmonary function testing, thoracic HRCT imaging, and transthoracic echocardiography (TTE). All were seen at National Jewish Medical and Research Center between

Results

Twenty four of 41 subjects (59%) had an abnormal pericardium by HRCT. Twenty of 41 subjects (49%) had a PEf, 23 of 41 subjects (56%) had an abnormal APR, and 20 of 41 subjects (49%) had an abnormal TPS.

An abnormal pericardium was more common in men than women. Subjects with and without pericardial abnormalities were otherwise similar with respect to age, SSc classification, autoantibodies, ILD pattern, presence of esophageal dilation, and pulmonary physiology (Table 1). Subjects with

Discussion

SSc patients commonly have asymptomatic pericardial abnormalities (reviewed by Deswal and Follansbee8), with autopsy series reporting a prevalence of 33 to 72%.8910111213 Fibrinous pericarditis, fibrous pericarditis, pericardial adhesions, and pericardial effusions have all been described.11 Screening echocardiographic evaluations have shown a prevalence of asymptomatic PEf of 13 to 55%.814151617 The prevalence of symptomatic pericardial abnormalities is much less prevalent, with studies8101112

References (27)

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None of the authors have any financial or other potential conflicts of interest to disclose.

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