Disease stratification in idiopathic pulmonary fibrosis: the dawn of a new era?

Maher, Toby M.

doi:10.1183/09031936.00228513

Cited by 8 publications

(3 citation statements)

References 25 publications

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“…Usual interstitial pneumonia is spatially heterogeneous, both macroscopically and microscopically, demonstrating a peripheral and basal predominant distribution. Despite recently available therapies that appear to slow the rate of disease progression (3,4), current measures of disease severity are insensitive and nonspeciic, prompting searches for clinical and imaging biomarkers of disease to assist with phenotyping, prognostication, and the development of therapies (5,6).…”

mentioning

confidence: 99%

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI

Chan¹,

Weatherley²,

Johns³

et al. 2019

Radiology

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mentioning

confidence: 99%

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI

Chan¹,

Weatherley²,

Johns³

et al. 2019

Radiology

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“…The identification of subgroups of patients more likely to respond to a compound's mechanism of action (so called "predictive" cohort enrichment) may allow for the detection of significant differences in biological end-points in relatively small sample sizes [12][13][14]. Additional study design approaches that enhance trial efficiency such as crossover groups, trials with multiple periods of comparison, and analytical methods that use average rank scores considering multiple end-points can also greatly enhance study power [15][16][17][18].…”

Section: Establishment Of Expectations For Early Phase Proof Of Concementioning

confidence: 99%

A new era in idiopathic pulmonary fibrosis: considerations for future clinical trials

et al. 2015

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The past decade has seen substantial progress in understanding the pathobiology, natural history, and clinical significance of idiopathic pulmonary fibrosis (IPF), culminating in the establishment of two effective medical therapies. Now seems an important time to reconsider the design and conduct of future IPF clinical trials. Building on lessons learned over the past decade, we use this perspective to lay out four key considerations for moving forward effectively and efficiently with the next generation of clinical trials in IPF. These are: development of a coordinated IPF clinical trials network; establishment of expectations for early phase proof of concept studies; adaptation of late-phase efficacy trial designs to the emergence of approved therapies, and; agreement on primary end-points for late phase clinical trials. Continued progress in the field of IPF will require creativity and collaboration on the part of all stakeholders. We believe that addressing these four considerations will encourage and enable investment in this new era of drug development in IPF, and will lead to more rapid development of effective therapies. @ERSpublications What are the key considerations for the next generation of clinical trials in idiopathic pulmonary fibrosis? http://ow.ly/I0TUc

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“…Additionally, identifying IPF patients with a prothrombotic state would allow anticoagulation to be targeted to those most likely to benefit. This therapeutic approach may be desirable in IPF [37]. Conducting large clinical trials of individualised therapies in IPF subgroups would require global collaboration in order to achieve adequate statistical power, supporting the need for a global IPF registry [38].…”

Section: The Coagulation Cascade and Fibrinolysismentioning

confidence: 99%

Coagulation and anticoagulation in idiopathic pulmonary fibrosis

Crooks

Hart

2015

Eur Respir Rev

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Idiopathic pulmonary fibrosis (IPF) is an incurable, progressive interstitial lung disease with a prognosis that is worse than that of many cancers. Epidemiological studies have demonstrated a link between IPF and thrombotic vascular events. Coagulation and fibrinolytic systems play central roles in wound healing and repair, processes hypothesised to be abnormal within the IPF lung. Animal models of pulmonary fibrosis have demonstrated an imbalance between thrombosis and fibrinolysis within the alveolar compartment, a finding that is also observed in IPF patients. A systemic prothrombotic state also occurs in IPF and is associated with increased mortality, but trials of anticoagulation in IPF have provided conflicting results. Differences in methodology, intervention and study populations may contribute to the inconsistent trial outcomes. The new oral anticoagulants have properties that may prove advantageous in targeting both thrombotic risk and progression of lung fibrosis. @ERSpublications A review of the coagulation system in IPF exploring scientific and clinical studies in this area

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Disease stratification in idiopathic pulmonary fibrosis: the dawn of a new era?

Cited by 8 publications

References 25 publications

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI

A new era in idiopathic pulmonary fibrosis: considerations for future clinical trials

Coagulation and anticoagulation in idiopathic pulmonary fibrosis

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Disease stratification in idiopathic pulmonary fibrosis: the dawn of a new era?

Cited by 8 publications

References 25 publications

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized 3He Diffusion-weighted MRI

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized 3He Diffusion-weighted MRI

A new era in idiopathic pulmonary fibrosis: considerations for future clinical trials

Coagulation and anticoagulation in idiopathic pulmonary fibrosis

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Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI

Airway Microstructure in Idiopathic Pulmonary Fibrosis: Assessment at Hyperpolarized ³He Diffusion-weighted MRI