Original clinical science
Variable prevalence of pulmonary hypertension in patients with advanced interstitial pneumonia

https://doi.org/10.1016/j.healun.2009.07.025Get rights and content

Background

Pulmonary hypertension may occur in patients with interstitial pneumonia and is associated with increased mortality. We sought to determine the prevalence of pulmonary hypertension in sub-groups of patients with interstitial pneumonia and to investigate possible associations between pulmonary vascular hemodynamics and pulmonary function.

Methods

The presence or absence of pulmonary hypertension was assessed in 70 patients with advanced interstitial pneumonia who underwent right heart catheterization. The associations of pulmonary hypertension with clinical characteristics and pulmonary function tests were analyzed.

Results

The prevalence of pulmonary hypertension in patients with idiopathic interstitial pneumonia was 29% vs 64% in patients with connective tissue disease-interstitial pneumonia (p = 0.013). African American patients had a significantly higher prevalence of pulmonary hypertension in the entire study population (81% vs 22%, p < 0.001) and in the idiopathic interstitial pneumonia group (70% vs 19%, p < 0.01). Regression analyses revealed no association between mean pulmonary artery pressure (mPAP) and forced vital capacity or mPAP and diffusion capacity of the lung for carbon monoxide in the entire cohort or in sub-groups of patients.

Conclusions

African American patients and patients with connective tissue disease-interstitial pneumonia had a high prevalence of pulmonary hypertension. Non-African American patients with advanced idiopathic interstitial pneumonia (including idiopathic pulmonary fibrosis) had a low prevalence of pulmonary hypertension.

Section snippets

Patients

The study was reviewed and approved by the University of Maryland Institutional Review Board. Patients with one of the forms of IIP or CTD-IP were identified at the University of Maryland Medical Center through patient care visits or through International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) code review for “interstitial lung disease.” Patients with sarcoidosis were excluded because the clinical course, pulmonary physiology, and lung pathology of

Patient characteristics

Initially, 142 patients were identified at the University of Maryland Medical Center through patient care visits or through ICD-9CM code review for “interstitial lung disease.” From this initial group, 70 patients were evaluable and enrolled in the current study because all of the following criteria were met: (1) RHC was performed; (2) CT scans of the chest were obtained; and (3) review of the clinical history, PFTs, chest CT, and lung pathology allowed a consensus for a final clinical

Discussion

In this study, we addressed the hypothesis that the prevalence of pulmonary hypertension may vary among patients with interstitial pneumonia depending on their underlying diagnosis, their race, or other clinical characteristics. The prevalence of pulmonary hypertension in patients with IIP was 29%, and the prevalence of pulmonary hypertension in the sub-group of these patients with IPF was also 29%. This observed prevalence is comparable with the reports of other groups.4, 6, 7 We found a

Disclosure Statement

This study was supported by National Institutes of Health R01HL054163, R01HL074067, and VA Merit Review (all to SPA).

None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.

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