Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderate restriction

Raghu, Ganesh; Nathan, Steven D.; Behr, Jüergen; Brown, Kevin K.; Egan, J. J.; Kawut, Steven M.; Flaherty, Kevin R.; Martínez, Fernando J.; Wells, Adrian; Shao, Lixin; Hong, Zhou; Henig, Noreen R.; Szwarcberg, Javier; Gillies, Hunter; Montgomery, A. Bruce; O’Riordan, Thomas G.

doi:10.1183/13993003.01537-2014

Cited by 140 publications

(149 citation statements)

References 16 publications

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“…Patients with PH and normal capillary wedge pressure had lower DLCO and increased haemoglobin desaturation at exercise. Interestingly, PH does not seem to progress rapidly in these patients, as repeat catheterisation at 48 weeks yielded levels quite similar to baseline [87].…”

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 93%

“…There is no correlation between mPAP and operating lung volumes [87,91]. Anatomical-functional correlation analysis in 26 explanted lungs showed that mPAP is significantly correlated with the extent of fibrosis in damaged regions, but no correlation exists between mPAP and the extent of venous lesions in less-damaged areas [28].…”

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 94%

“…In a series of 488 IPF patients with minimal honeycomb changes (<5% of the lung) and mild-to-moderate restriction (mean FVC between 67% and 69% pred), right-heart catheterisation showed PH without elevated capillary wedge pressure in 14% of patients, PH with elevated wedge pressure in 5%, and isolated elevated wedge pressure in 4% [87]. Patients with PH and normal capillary wedge pressure had lower DLCO and increased haemoglobin desaturation at exercise.…”

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 99%

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Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed.

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Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 93%

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 94%

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 99%

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Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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“…Much less is known about the natural cause of pulmonary hypertension in idiopathic pulmonary fibrosis (IPF), because most studies are limited by their focus on advanced lung disease. Recently, RAGHU et al [33] filled this gap using right heart catheterisation data from 488 subjects enrolled in a placebo-controlled study of ambrisentan in IPF with mild-moderate lung volume restriction. As in COPD, it was shown that severe pulmonary hypertension is rare in IPF and that pulmonary artery pressure remains stable over a 1-year period in the majority of IPF patients.…”

Section: Pulmonary Hypertension Secondary To Pulmonary Diseasementioning

confidence: 99%

Pulmonary hypertension

2016

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In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summarise recent advances in pulmonary vasculature science. The selection we made from the enormous amount of published work undoubtedly reflects our personal views and may not include all papers with a significant impact in the near or more distant future. The focus of this paper is on the diagnosis of pulmonary arterial hypertension, understanding the success of combination therapy on the right ventricle and scientific breakthroughs. @ERSpublicationsThe review summarises advances in pulmonary hypertension since the publication of the recent ESC/ERS guidelines http://ow.ly/WUwoeThe global picture of pulmonary arterial hypertension Table 1 summarises the recent classification of pulmonary hypertension [1,2]. Based on data from the large European and North American registries, the most common types of pulmonary arterial hypertension (PAH) are idiopathic PAH and PAH associated with connective tissue disease. Less is known of the epidemiology of PAH in other parts of the world. Using the data from a large reference centre in Brazil, ALVES et al. [3] showed that schistosomiasis is among the top three of causes of PAH in that country. These global epidemiological data emphasise the importance of accounting for such differences in future clinical trials. Pulmonary veno-occlusive diseaseAn important change from the previous classification is that significant progress has been made in the field of pulmonary veno-occlusive disease (PVOD). Several causes of PVOD have been identified in recent years, including genetics, drugs and radiation therapy. The finding of the EIF2AK4 (eukaryotic translation initiation factor 2α kinase 4) mutation in familial PVOD and pulmonary capillary haemangiomatosis (PCH) might boost further research [4,5]. By the discovery of this gene, it is possible to confirm the diagnosis of PVOD or PCH by demonstrating the presence of the mutation instead of a histological diagnosis [5]. Of interest is the recent study by PERROS et al. [6] showing not only that mitomycin is a risk factor for the development of PVOD, but also that mitomycin induces pulmonary vascular disease in rats that resembles the pathological features of PVOD. This finding not only offers a representative animal model to study the disease but also sheds new light on the possible role of alkylating chemotherapy on the development of pulmonary hypertension [7]. New associations between drugs and disease were not only made in PVOD; in PAH, a possible relationship between a drug and the disease also was found. A recent

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“…IPF clinical trials using vasodilators such as the endothelin receptor antagonists have thus far been negative [14][15][16]. It appears, however, that sildenafil has pleiotropic properties beyond its traditional vasodilatory effects that may render it especially attractive as an add-on treatment for IPF.…”

mentioning

confidence: 99%