Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis

Nadrous, Hassan F.; Pellikka, Patricia A.; Krowka, Michael J.; Swanson, Karen L.; Chaowalit, Nithima; Decker, Paul A.; Ryu, Jay H.

doi:10.1378/chest.126.2.438

Cited by 99 publications

(85 citation statements)

References 51 publications

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“…In CVDs, it was reported that PH at rest was frequently found in patients with MCTD and SSc (7 to 50%) (5,8), whereas PH at rest was reported to be rare in PM / DM, RA, and in Sjögren's syndrome (6,7). The finding of significant association of PH with DLCO in IIPs was consistent with the report of Nadrous et al (2), who showed that sPAP at rest was inversely correlated with DLCO and PaO2 in patients with IPF/UIP. In a previous study in our institution, it was also shown that the mean pulmonary artery pressure measured by right heart catheterization negatively correlated with %DLCO in patients with IPF/UIP (25).…”

Section: Pulmonary Function Testssupporting

confidence: 90%

“…, and the condition is as-sociated with a poor prognosis (2). PH could be a causative factor that drives the process of IPF into acute exacerbation.…”

Section: Idiopathic Interstitial Pneumonias (Iips) Make Up a Heterogementioning

confidence: 99%

“…1 Table 5). (2). In CVDs, it was reported that PH at rest was frequently found in patients with MCTD and SSc (7 to 50%) (5,8), whereas PH at rest was reported to be rare in PM / DM, RA, and in Sjögren's syndrome (6,7).…”

Section: Pulmonary Function Testsmentioning

confidence: 99%

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Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

et al. 2007

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Section: Pulmonary Function Testssupporting

confidence: 90%

“…, and the condition is as-sociated with a poor prognosis (2). PH could be a causative factor that drives the process of IPF into acute exacerbation.…”

Section: Idiopathic Interstitial Pneumonias (Iips) Make Up a Heterogementioning

confidence: 99%

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Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

et al. 2007

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“…There is a known, but imperfect, inverse correlation between D lco % predicted and PH. 21 Nadrous et al 22 previously reported on 88 patients with IPF who were stratifi ed by RVSP. Forty-seven subjects had an RVSP 35-50 mm Hg (mean, 43 mm Hg), which is comparable to our series.…”

Section: Discussionmentioning

confidence: 99%

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

Han

Bach

Hagan

et al. 2013

Chest

203

155

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Background: Idiopathic pulmonary fi brosis (IPF) is a progressive lung disease with pulmonary vasculopathy. Objective: The purpose of this study was to determine whether sildenafi l improves 6-min walk distance (6MWD) in subjects with IPF and right ventricular dysfunction. Methods: The IPFnet, a network of IPF research centers in the United States, conducted a randomized trial examining the effect of sildenafi l on 6MWD in patients with advanced IPF, defi ned by carbon monoxide diffusing capacity , 35% predicted. A substudy examined 119 of 180 randomized subjects where echocardiograms were available for independent review by two cardiologists. Right ventricular (RV) hypertrophy (RVH), right ventricular systolic dysfunction (RVSD), and right ventricular systolic pressure (RVSP) were assessed. Multivariable linear regression models estimated the relationship between RV abnormality, sildenafi l treatment, and changes in 6MWD, St. George's Respiratory Questionnaire (SGRQ), the EuroQol instrument, and SF-36 Health Survey (SF-36) from enrollment to 12 weeks. Results: The prevalence of RVH and RVSD were 12.8% and 18.6%, respectively. RVSP was measurable in 71 of 119 (60%) subjects; mean RVSP was 42.5 mm Hg. In the subgroup of subjects with RVSD, subjects treated with sildenafi l experienced less decrement in 6MWD (99.3 m; P 5 .01) and greater improvement in SGRQ (13.4 points; P 5 .005) and EuroQol visual analog scores (17.9 points; P 5 .04) than subjects receiving placebo. In the subgroup with RVH, sildenafi l was not associated with change in 6MWD ( P 5 .13), but was associated with greater relative improvement in SGRQ (14.8 points; P 5 .02) vs subjects receiving placebo. Sildenafi l treatment in those with RVSD and RVH was not associated with change in SF-36. Conclusions: Sildenafi l treatment in IPF with RVSD results in better preservation of exercise capacity as compared with placebo. Sildenafi l also improves quality of life in subjects with RVH and RVSD.CHEST 2013; 143(6):1699-1708Abbreviations: 6MWD 5 6-min walk distance; BNP 5 brain natriuretic peptide; D lco 5 carbon monoxide diffusing capacity; IPF 5 idiopathic pulmonary fi brosis; PAH 5 pulmonary arterial hypertension; PH 5 pulmonary hypertension; QOL 5 quality of life; RHC 5 right-sided heart catheterization; RV 5 right ventricular; RVH 5 right-sided ventricular hypertrophy; RVSD 5 right-sided ventricular systolic dysfunction; RVSP 5 right-sided ventricular systolic pressure;

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“…38 The systolic pulmonary artery pressure (sPAP), estimated by echocardiography, strongly correlates with survival. 35 Patients with an estimated sPAP greater than 50 mm Hg had a median survival of only 0.7 years versus more than 4 years for those patients with an sPAP of less than 50 mm Hg. In a more recent study, 79 IPF patients underwent right-heart catheterization.…”

Section: What Are the Prognostic Factors Associated With Idiopathic Pmentioning

confidence: 93%

Spectrum of Fibrosing Diffuse Parenchymal Lung Disease

Morgenthau

Padilla

2009

Mount Sinai J Medicine

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The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky‐Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end‐stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed. Mt Sinai J Med 76:2–23, © 2009 Mount Sinai School of Medicine

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Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis

Cited by 99 publications

References 51 publications

Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

Spectrum of Fibrosing Diffuse Parenchymal Lung Disease

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