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A disease in which the alveoli (tiny air sacs at the end of the bronchioles in the lungs) are overgrown with fibrous tissue. The cause of the disease is unknown and it gets worse over time. Symptoms include difficult, painful breathing and shortness of breath.

Results: 1 to 20 of 59

Currently there is no evidence to support the routine use of corticosteroids alone in the management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic disorder of unknown cause that is progressive and leads to death. Existing evidence about the treatment of IPF is difficult to interpret because of evolving diagnostic criteria and classification schemes, grouping together diverse entities with markedly different natural histories and responses to therapy. Therefore, because the old studies did not include only IPF patients, their results cannot be extended to IPF as they are currently defined. In addition, methodologic problems mainly related to the lack of randomized placebo‐controlled treatment trials have made evidence‐based conclusions difficult. A systematic search for high quality placebo‐controlled randomised trials comparing corticosteroid treatment with placebo in subjects with a diagnosis of IPF was unable to identify any studies suitable for inclusion in the review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2010

Non‐steroid agents for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a form of progressive lung disease which ultimately leads to death. The cause is unknown, but the disease is characterised by scar tissue in the lungs. This prevents the lungs from working effectively. Standard treatment uses oral corticosteroids in association with immunosuppressors, but there is uncertainty as to whether this treatment is effective. Immunosuppressive agents such as azathioprine and cyclophosphamide have been used to treat the disease because it is thought they might prevent inflammation. The review found 15 high quality trials of non‐steroid drugs tested in idiopathic pulmonary fibrosis patients. Notwithstanding the encouraging results of a first small study included in the first version of this review, the effects of interferon gamma‐1beta, as assessed by combining two subsequent large trials, were disappointing and failed to show an effect on improving survival. Four studies did evaluate pirfenidone, an anti‐fibrotic oral drug, on a large number of patients: although two of these studies have only been presented in conferences,combining the published and unpublished data showed a significant improvement of pirfenidone on progression‐free survival and a small increase in pulmonary function. Current evidence suggests a possible role for pirfenidone in the treatment of idiopathic pulmonary fibrosis, though data on survival are now needed. However, trials with other non‐steroid agents are currently ongoing and new evidence may become available soon.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2010

Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis: Idiopathic Pulmonary Fibrosis [Internet]

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease (ILD) of unknown origin. It is a difficult disease to diagnose and often requires the collaborative expertise of a chest physician, radiologist and histopathologist to reach a consensus diagnosis. Most people with idiopathic pulmonary fibrosis experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death. Specific pharmacological therapies for IPF are limited but the last decade has seen more trials of new drugs which have had a variable impact on clinical practice. A number of difficulties arise when undertaking clinical trials in IPF in terms of defining precise, diagnostic inclusion criteria and clinically meaningful end-points. However, such trials are the only way by which promising new treatments will come to benefit patients. Furthermore, it is only by performing rigorous clinical trials, we have learned that drugs once widely used to treat IPF may in fact have been harmful. The limitations of current pharmacological therapies for IPF highlight the importance of other forms of treatment including lung transplantation and best supportive care such as oxygen therapy, pulmonary rehabilitation and palliation of symptoms. These are interventions which justifiably require scrutiny in the context of healthcare delivery by the modern NHS. Despite the significant burden of disease caused by IPF, there is currently no established framework within the NHS for its diagnosis and management thus creating an environment in which significant variations in clinical care may occur. In recognition of this, the Department of Health commissioned the National Institute of Health and Care Excellence (NICE) to produce a guideline aimed at improving the care of people with IPF.

NICE Clinical Guidelines - National Clinical Guideline Centre (UK).

Version: June 2013
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The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

The study found that few interventions for idiopathic pulmonary fibrosis have any statistically significant effect, and identified a lack of studies on palliative care approaches. Few direct comparisons of treatments were identified; an indirect comparison was performed through network meta-analysis, but the results should be interpreted with caution.

Health Technology Assessment - NIHR Journals Library.

Version: March 2015
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Efficacy of N-acetylcysteine for patients with idiopathic pulmonary fibrosis: a meta-analysis

Bibliographic details: Fu XW, Tong X, Liu ST, Fan H.  Efficacy of N-acetylcysteine for patients with idiopathic pulmonary fibrosis: a meta-analysis. Chinese Journal of Evidence-Based Medicine 2014; 14(4): 449-455 Available from: http://dx.doi.org/10.7507/1672-2531.20140078

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2014

Interferon-gamma1b therapy in idiopathic pulmonary fibrosis: a metaanalysis

CONTEXT: Despite the investigation of multiple therapeutic options, idiopathic pulmonary fibrosis (IPF) remains a devastating, progressively fatal disease. Much interest has focused on the use of interferon (IFN)-gamma1b therapy, but the efficacy of this treatment has not been proven.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2005

Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall, between single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and summarize wait-list survival.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2014

The effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: systematic review, network meta-analysis and health economic evaluation

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease with considerable impact on patients and carers as the disease progresses. Currently few treatments are available. We aimed to evaluate the clinical and cost-effectiveness of available treatments for IPF.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2014

Use of oxygen during activities for people with lung scarring conditions

Many people with ILD have a drop in their oxygen levels when they are moving about. This is because of their lung scarring. Oxygen can be given, to be used only when people are moving about, to prevent this drop. This is called ambulatory oxygen. This may help to improve symptoms of breathlessness and their ability to exercise. It may also improve their quality of life. This Cochrane review examined the evidence for any effect of ambulatory oxygen on the ability to exercise, breathlessness and quality of life. Cochrane researchers searched the available evidence up to 4th May 2016.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Dyspnea in idiopathic pulmonary fibrosis: a systematic review

This review concluded that sildenafil and pulmonary rehabilitation are potential therapies for dyspnoea in selected patients with idiopathic pulmonary fibrosis. Additional research was suggested to examine the effects of supplemental oxygen and opioids. These conclusions reflect some of the evidence presented; limitations of the small evidence base mean that the recommendations for further research are justified.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2012

Effectiveness of Recombinant Human Growth Hormone (rhGH) in the Treatment of Patients With Cystic Fibrosis [Internet]

This is an evidence report prepared by the University of Connecticut/Hartford Hospital Evidence-based Practice Center (EPC) examining the benefits and harms associated with using recombinant human growth hormone (rhGH) in patients with cystic fibrosis (CF).

Comparative Effectiveness Reviews - Agency for Healthcare Research and Quality (US).

Version: October 2010
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Pulmonary rehabilitation for interstitial lung disease (ILD)

Review question: We reviewed available evidence on the effects of pulmonary rehabilitation on exercise capacity, shortness of breath and quality of life in people with interstitial lung disease (ILD).

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Drugs for Pulmonary Arterial Hypertension: Comparative Efficacy, Safety, and Cost-Effectiveness [Internet]

The objective of this therapeutic review is to conduct a systematic review to assess the comparative efficacy and safety and to determine the cost-effectiveness of drug therapies for the treatment of pulmonary arterial hypertension (PAH) in adults.

CADTH Therapeutic Review - Canadian Agency for Drugs and Technologies in Health.

Version: March 2015
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Macitentan (Opsumit): For Long-Term Treatment of Pulmonary Arterial Hypertension [Internet]

Pulmonary arterial hypertension (PAH; also classified as Group 1 pulmonary hypertension [PH]) is a rare, debilitating, progressive, and life-threatening disease of the pulmonary vasculature, characterized by vascular proliferation and remodelling of small pulmonary arteries. PAH is defined by an increase in mean pulmonary arterial pressure (mPAP) ≥ 25 mm Hg and a pulmonary wedge pressure of ≤ 15 mm Hg. If left untreated, it can lead to right heart failure and premature death.

Common Drug Review - Canadian Agency for Drugs and Technologies in Health.

Version: July 2015
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Riociguat (Adempas): Management of Inoperable Chronic Thromboembolic Pulmonary Hypertension [Internet]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease subtype of pulmonary hypertension. It is a progressive disease characterized by the presence of non-resolving or recurrent thrombi distributed within the pulmonary arteries. This can obstruct or occlude the luminal space, eventually leading to increased pulmonary vascular resistance (PVR), pulmonary hypertension, and right-sided heart failure. Although the exact etiology of CTEPH remains poorly understood, it may arise following an initial episode of acute pulmonary embolism (PE); however, up to 60% of CTEPH patients have not had any antecedent episode of acute PE. The epidemiology of CTEPH is likewise not well established. Some surveillance data estimate CTEPH to occur in 0.1% to 0.5% of patients surviving an initial episode of acute PE; however, the true incidence of CTEPH is likely to be higher, owing to an unknown number of undetected cases either presenting occultly or latently in the setting of acute PE or through non-venous thromboembolism etiologies.

Common Drug Review - Canadian Agency for Drugs and Technologies in Health.

Version: July 2015
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Optimizing Health System Use of Medical Isotopes and Other Imaging Modalities [Internet]

The purpose of this project was to provide national guidance on the optimal use of 99mTc during a situation of reduced supply. To accomplish this, our objective at CADTH was:

Optimal Use Report - Canadian Agency for Drugs and Technologies in Health.

Version: 2012

Benzodiazepines for the relief of breathlessness in advanced diseases in adults

Breathlessness is a common and distressing symptom in advanced cancer and other diseases at the end of life. Treating breathlessness sufficiently remains very difficult. Benzodiazepines are a group of sedating medicines (drugs), including lorazepam, clorazepate, diazepam, alprazolam, and temazepam, that are used mainly for sleep disturbance and anxiety, but are widely used for the relief of breathlessness.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Recombinant human growth hormone in the treatment of patients with cystic fibrosis

CONTEXT: Recombinant human growth hormone (rhGH) improves growth in patients with growth hormone deficiency or idiopathic short stature. Its role in patients with cystic fibrosis (CF) is unclear.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2010

The use of exploratory analyses within the National Institute for Health and Care Excellence single technology appraisal process: an evaluation and qualitative analysis

The study found that independent Evidence Review Groups frequently conduct exploratory analyses to test or improve the economic evaluations submitted to the National Institute for Health and Care Excellence (NICE) by companies as part of the single technology appraisal process. These analyses often influence the recommendations produced by NICE Technology Appraisal Committees.

Health Technology Assessment - NIHR Journals Library.

Version: April 2016
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The use of fibrin sealant during non-emergency surgery: a systematic review of evidence of benefits and harms

This study found that the effectiveness of fibrin sealants do not appear to vary according to surgical procedures with regard to reducing the risk of seroma or haematoma.

Health Technology Assessment - NIHR Journals Library.

Version: December 2016

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