Bosentan in Pulmonary Hypertension Associated with Fibrotic Idiopathic Interstitial Pneumonia

Corte, Tamera J.; Keir, Gregory J.; Dimopoulos, Konstantinos; Howard, Luke; Corris, Paul A.; Parfitt, Lisa; Foley, Claire; Yanez-Lopez, Monica; Babalis, Daphne; Marino, Philip; Maher, Toby M.; Renzoni, Elisabetta A.; Spencer, Lisa; Elliot, Charlie; Birring, Surinder S.; O’Reilly, Katherine Ma; Gatzoulis, Michael Α.; Wells, Adrian; Wort, Stephen J.

doi:10.1164/rccm.201403-0446oc

Cited by 188 publications

(183 citation statements)

References 33 publications

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“…The development of PH in ILD is a strong predictor of poor survival [46,56,57]. Unfortunately, the use of anti-PH treatments in this group of patients has not generated much optimism to date, as outlined in the previous sections and by a recent placebo-controlled trial of bosentan in patients with idiopathic interstitial pneumonias and PH [58]. The mechanisms thought to contribute to PH in ILD vary, and include hypoxic vasoconstriction, vascular remodelling, vessel ablation, vascular occlusion and compression.…”

Section: Nocturnal Hypoxia and Supplemental Oxygen In Ipfmentioning

confidence: 99%

Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

et al. 2015

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The prevalence of obstructive sleep apnoea (OSA) is continuously increasing in patients with idiopathic pulmonary fibrosis (IPF) and, for the first time, the recent IPF guidelines recognise OSA as an important associated comorbidity that can affect patient's survival. Thus, it becomes conceivable that clinicians should refer patients with newly diagnosed IPF to sleep centres for the diagnosis and treatment of OSA as well as for addressing issues regarding the reduced compliance of patients with continuous positive airway pressure therapy. The discovery of biomarkers common to both disorders may help early diagnosis, institution of the most appropriate treatment and follow-up of patients. Better understanding of epigenetic changes may provide useful information about pathogenesis and, possibly, development of new drugs for a dismal disease like IPF. @ERSpublications It is now believed that IPF and sleep disorders can coexist in the same patient

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Section: Nocturnal Hypoxia and Supplemental Oxygen In Ipfmentioning

confidence: 99%

Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

et al. 2015

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“…The primary outcome was the fall of pulmonary vascular resistance by 20% or more over 16 weeks. The study was negative [61].…”

Section: Treatment Considerations Of Ph In the Course Of Dpldsmentioning

confidence: 90%

“…Corte et al didn't observe any significant difference in the oxygen saturation and oxygen requirement between bosentan and placebo groups in the patients with IPF and fibrotic NSIP [61]. Zisman et al found improved oxygenation in the group of IPF patients treated with Sildenafil in the STEP-IPF study [57].…”

Section: Treatment Considerations Of Ph In the Course Of Dpldsmentioning

confidence: 99%

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

Szturmowicz¹,

Kacprzak²,

Błasińska‐Przerwa³

et al. 2015

Advances in Respiratory Medicine

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Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both "parenchymal" and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.

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“…An earlier report from the same study showed that treatment with ambrisentan is not effective in these patients and is even associated with shorter time to disease progression [34]. In a later placebo-controlled trial by CORTE et al [35], patients with IPF and pulmonary hypertension were randomised to bosentan or placebo. At a 16-week follow-up, no difference was found between the placebo and treatment group in pulmonary haemodynamics, symptoms and functional capacity in patients with idiopathic interstitial pneumonia and pulmonary hypertension.…”

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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Bosentan in Pulmonary Hypertension Associated with Fibrotic Idiopathic Interstitial Pneumonia

Cited by 188 publications

References 33 publications

Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

Pulmonary hypertension

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