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<script type="text/javascript" src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/jr.boots.min.js"> </script><title>Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis - NCBI Bookshelf</title>
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<meta name="author" content="National Clinical Guideline Centre (UK)">
<meta name="citation_title" content="Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis">
<meta name="citation_publisher" content="Royal College of Physicians (UK)">
<meta name="citation_date" content="2013/06">
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<meta name="DC.Title" content="Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis">
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<meta name="description" content="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.">
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<meta name="og:description" content="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.">
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<meta name="warning" content="May 2017: Recommendation 27 was amended by NICE to add a link to the NICE technology appraisal on nintedanib for the treatment of idiopathic pulmonary fibrosis. Two outdated research recommendations have been stood down and removed from the short version. In this version they have been greyed out in the list of Key Research Recommendations.">
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Two outdated research recommendations have been stood down and removed from the short version. In this version they have been greyed out in the list of Key Research Recommendations.</strong></p><div class="main-content lit-style"><div class="fm-sec bkr_bottom_sep"><div class="bkr_thumb"><a href="http://www.rcplondon.ac.uk/Pages/index.aspx" title="Royal College of Physicians (UK)" class="img_link icnblk_img" ref="pagearea=logo&amp;targetsite=external&amp;targetcat=link&amp;targettype=publisher"><img class="source-thumb" src="/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-nicecg163-lrg.png" alt="Cover of Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis" /></a></div><div class="bkr_bib"><h1 id="_NBK247530_"><span itemprop="name">Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis</span></h1><div class="subtitle">Idiopathic Pulmonary Fibrosis</div><p><i>NICE Clinical Guidelines, No. 163</i></p><p class="contrib-group"><h4>Authors</h4><span itemprop="author">National Clinical Guideline Centre (UK)</span>.</p><div class="half_rhythm">London: <a href="http://www.rcplondon.ac.uk/Pages/index.aspx" ref="pagearea=meta&amp;targetsite=external&amp;targetcat=link&amp;targettype=publisher"><span itemprop="publisher">Royal College of Physicians (UK)</span></a>; <span itemprop="datePublished">2013 Jun</span>.</div><div><a href="/books/about/copyright/">Copyright</a> &#x000a9; 2013, National Clinical Guideline Centre.</div><a class="btn wsprkl bkr_rd" href="/books/n/nicecg163/ack/?report=reader">Read</a></div><div class="bkr_clear"></div></div><div class="bkr_bottom_sep bkr_bottom_margin body-content whole_rhythm"><div itemprop="description"><h2>Excerpt</h2><p>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.</p></div></div></div><div class="fm-sec"><div><b>Funding</b>: National Institute for Health and Care Excellence</div><div><p><b>Disclaimer</b>: Healthcare professionals are expected to take NICE clinical guidelines fully into account when exercising their clinical judgement. However, the guidance does not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of each patient, in consultation with the patient and/or their guardian or carer.</p></div><div class="half_rhythm"><a href="/books/about/copyright/">Copyright</a> &#x000a9; 2013, National Clinical Guideline Centre.</div><div class="small"><span class="label">Bookshelf ID: NBK247530</span><span class="label">PMID: <a href="https://pubmed.ncbi.nlm.nih.gov/25340234" title="PubMed record of this title" ref="pagearea=meta&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">25340234</a></span></div></div><div class="small-screen-prev"></div><div class="small-screen-next"></div></article></div><div id="jr-scripts"><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/libs.min.js"> </script><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/jr.min.js"> </script></div></div>
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