Original clinical scienceVariable prevalence of pulmonary hypertension in patients with advanced interstitial pneumonia
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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Cited by (25)
Pulmonary hypertension associated with lung disease: new insights into pathomechanisms, diagnosis, and management
2023, The Lancet Respiratory MedicinePulmonary hypertension in chronic obstructive and interstitial lung diseases
2013, International Journal of CardiologyCitation Excerpt :Low arterial oxygenation, and a poor 6MWT [25] should increase suspicion of PH [26–29]. African–Americans seem to have a higher propensity for PH [30]. There is an association between a low diffusion capacity (Dlco) [26] and the presence of PH in COPD [31] and ILD [29].
Classification of Pulmonary Hypertension
2012, Heart Failure ClinicsCitation Excerpt :Numerous studies have indicated that even mild forms of PH may be clinically significant in patients with COPD as well as in patients with pulmonary fibrosis, as they are associated with worse oxygenation, decreased exercise capacity, and poorer prognosis.101,107–110 Of the parenchymal lung diseases, ILD most commonly results in PH through capillary destruction and hypoxic vasoconstriction.111,112 Patients with CTD, in particular those with SSc of CTD, are at increased risk for the development of ILD and secondary PH. Occasionally such patients will develop coexistent pulmonary vascular disease (PAH), and it is important to distinguish as much as possible between PAH and PH related to ILD, because none of the current therapies for PAH have been shown to be safe and effective in treating PH related to ILD.
Pulmonary hypertension in interstitial lung disease: Prevalence, prognosis and 6 min walk test
2012, Respiratory MedicineCitation Excerpt :For example, in consecutive sarcoidosis patients, 5.7% had PH based on echocardiography,6 while a prevalence of 48% was reported in IPF transplant candidates.5 A number of studies have investigated lung transplant candidates in whom RHC measurements are done routinely.4,5,7,23,24 But lung transplant candidates only constitute a small group of patients at referral centres (less than 4% at our institution), and as they have particularly advanced lung disease, the prevalence of PH may be notably higher than in ILD patients in general.
Interstitial lung disease: The initial approach
2011, Medical Clinics of North AmericaCitation Excerpt :However, the absence of crackles does not rule out ILD, particularly in the presence of extrapulmonary signs (Table 1). Pulmonary hypertension is frequently seen in patients with CTD-associated ILD,21–25 notably in scleroderma, polymyositis, mixed connective tissue disease, and occasionally rheumatoid arthritis, sarcoidosis,26–28 and IPF.29,30 Physical signs suggestive of pulmonary hypertension include increased jugular “a” and “v” waves, left parasternal lift, accentuated pulmonary component of the second heart sound, right ventricular S4, diastolic murmur of pulmonary regurgitation, holosytolic murmur of tricuspid regurgitation, and hepatojugular reflux indicating high central venous pressure.31