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{{Short description|Scarring of the lungs for no known reason}}
{{Use dmy dates|date=August 2022}}
{{Infobox medical condition (new)
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<!-- Definition and symptoms -->
'''Idiopathic pulmonary fibrosis''' ('''IPF''')
The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs.<ref>{{Cite web|title=Idiopathic Pulmonary Fibrosis | work = NHLBI, NIH|url= https://www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis |access-date=5 December 2020 }}</ref> Symptoms typically include gradual onset of [[dypsnea|shortness of breath]] and a dry [[cough]].<ref name=NIH2018>{{cite web|title=Idiopathic Pulmonary Fibrosis|url=http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/|website=NHLBI|access-date=21 January 2018}}</ref> Other changes may include feeling tired, and [[nail clubbing|clubbing]] abnormally large and dome shaped finger and toenails <!-- Cause and diagnosis -->
The cause is unknown, hence the term [[Idiopathic disease|idiopathic]].<ref name=ATS2015>{{cite journal | vauthors = Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J, Brozek JL, Collard HR, Cunningham W, Homma S, Johkoh T, Martinez FJ, Myers J, Protzko SL, Richeldi L, Rind D, Selman M, Theodore A, Wells AU, Hoogsteden H, Schünemann HJ | display-authors = 6 | title = An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline | journal = American Journal of Respiratory and Critical Care Medicine | volume = 192 | issue = 2 | pages = e3–19 | date = July 2015 | pmid = 26177183 | doi = 10.1164/rccm.201506-1063ST }}</ref> Risk factors include [[cigarette smoking]], [[Gastroesophageal reflux disease|acid reflux disease (GERD)]], certain [[viral infection]]s, and genetic predisposition.<ref name=NIH2018/> The underlying mechanism involves [[pulmonary fibrosis|scarring of the lungs]].<ref name=NIH2018/> Diagnosis requires ruling out other potential causes.<ref name=Rag2011 /> It may be supported by a [[High-resolution computed tomography|
<!-- Treatment -->
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<!-- Epidemiology and prognosis -->
About 5 million people are affected globally.<ref name=Melt2008>{{cite journal |vauthors=Meltzer EB, Noble PW | title = Idiopathic pulmonary fibrosis | journal = Orphanet Journal of Rare Diseases| volume = 3 | issue = 1 | pages = 8| year = 2008 | pmid = 18366757 | pmc = 2330030 | doi = 10.1186/1750-1172-3-8 | doi-access = free }}</ref> The disease newly occurs in about 12 per 100,000 people per year.<ref name=Fer2018/> Those in their 60s and 70s are most commonly affected.<ref name=Fer2018/> Males are affected more often than females.<ref name=Fer2018/> Average [[life expectancy]] following diagnosis is about four years.<ref name=NIH2018/> Updated international guidelines were published in 2022, which some simplification in diagnosis and the removal of antacids as a possible adjunct therapy.<ref>{{Cite journal |last1=Raghu |first1=Ganesh |last2=Remy-Jardin |first2=Martine |last3=Richeldi |first3=Luca |last4=Thomson |first4=Carey C. |last5=Inoue |first5=Yoshikazu |last6=Johkoh |first6=Takeshi |last7=Kreuter |first7=Michael |last8=Lynch |first8=David A. |last9=Maher |first9=Toby M. |last10=Martinez |first10=Fernando J. |last11=Molina-Molina |first11=Maria |last12=Myers |first12=Jeffrey L. |last13=Nicholson |first13=Andrew G. |last14=Ryerson |first14=Christopher J. |last15=Strek |first15=Mary E. |date=2022-05-01 |title=Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline |journal=American Journal of Respiratory and Critical Care Medicine |volume=205 |issue=9 |pages=e18–e47 |doi=10.1164/rccm.202202-0399ST |pmid=35486072 |pmc=9851481 |s2cid=248432749 |issn=1073-449X}}</ref>
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* Dry, non-productive cough on exertion
* Progressive exertional dyspnea (shortness of breath with exercise)
* Dry, inspiratory
* [[nail clubbing|Clubbing of the digits]], a disfigurement of the finger tips or toes (see image)
* Abnormal [[pulmonary function test]] results, with evidence of restriction and impaired [[gas exchange]].
Some of these features are due to chronic [[hypoxemia]] (oxygen deficiency in the blood), and are not specific for IPF,
Assessment of
If bilateral fine crackles are present throughout the inspiratory time and are persisting after several deep breaths, and if remaining present on several occasions several weeks apart in a subject aged ≥60 years, this should raise the suspicion of IPF and lead to consideration of an HRCT scan of the chest which is more sensitive than a [[chest X-ray]].<ref name=CottinCordier /> As crackles are not specific for IPF, they must prompt a thorough diagnostic process.<ref name=Rag2011 />
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IPF is believed to be the result of an aberrant wound healing process including/involving abnormal and excessive deposition of [[collagen]] (fibrosis) in the [[pulmonary interstitium]] with minimal associated [[inflammation]].<ref name=HarariCaminati>{{cite journal |vauthors=Harari S, Caminati A | title = IPF: new insight on pathogenesis and treatment | journal = Allergy| volume = 65 | issue = 5 | pages = 537–553 | year = 2010 | pmid = 20121758 | doi = 10.1111/j.1398-9995.2009.02305.x| s2cid = 21633787 | doi-access = free }}</ref> [[Cellular senescence]] is suspected to be a central contributing cause, a belief which is supported by benefits seen in patients given [[senolytic]] therapy.<ref name="pmid30616998">{{cite journal | vauthors = Justice JN, Nambiar AM, Tchkonia T, Kirkland JL | title = Senolytics in idiopathic pulmonary fibrosis: Results from a first-in-human, open-label, pilot study | journal = [[EBioMedicine]] | volume = 40 | pages = 554–563 | date=2019 | url= | doi = 10.1016/j.ebiom.2018.12.052 | pmc=6412088 | pmid = 30616998}}</ref><ref name="pmid31451866">{{cite journal | vauthors = Palmer AK, Gustafson B, Kirkland JL, Smith U | title = Cellular senescence: at the nexus between ageing and diabetes | journal = [[Diabetologia]] | volume = 62 | issue=10 | pages = 1835–1841 | date=2019 | url= | doi = 10.1007/s00125-019-4934-x | pmc=6731336 | pmid = 31451866}}</ref><ref name="pmid32686219">{{cite journal | vauthors = Kirkland JL, Tchkonia T| title = Senolytic Drugs: From Discovery to Translation | journal = [[Journal of Internal Medicine]] | date=2020 | volume = 288 | issue = 5 | pages = 518–536 | doi = 10.1111/joim.13141 | pmid = 32686219| pmc = 7405395 | doi-access = free }}</ref>
It is hypothesized that the initial or repetitive injury in IPF occurs to the lung
In the past, it was thought that [[inflammation]] was the first event in initiating lung tissue scarring. Later findings showed that the development of fibroblastic foci precedes the accumulation of inflammatory cells and the consequent deposition of collagen.<ref name="PardoSelman">{{cite journal |vauthors=Pardo A, Selman M | title = Idiopathic pulmonary fibrosis: new insights in its pathogenesis | journal = The International Journal of Biochemistry & Cell Biology| volume = 34 | issue = 12 | pages = 1534–1538 | year = 2002 | pmid = 12379275| doi = 10.1016/s1357-2725(02)00091-2}}</ref> This pathogenetic model is indirectly supported by the clinical features of IPF, including an insidious onset over several years, relatively infrequent acute exacerbations, and failure to respond to [[immunosuppressive therapy]].<ref name="HarariCaminati" /><ref name="SelmanKing">{{cite journal |vauthors=Selman M, King TE, Pardo A | title = Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy | journal = Annals of Internal Medicine| volume = 134 | issue = 2 | pages = 136–151 | year = 2001 | pmid = 11177318 | doi = 10.7326/0003-4819-134-2-200101160-00015| s2cid = 10955241 }}</ref> However, it is the belief of some researchers that the disease is a multi-mechanistic one, wherein the trigger for the disease may stem from abnormalities in any number of wound healing pathways, including the inflammatory response.<ref name=":0" /> Such abnormalities could occur in any number of the nine implicated pathways (clotting cascade, antioxidant pathways, apoptosis, inflammatory cytokines, angiogenesis and vascular remodelling, growth factors, surfactant and matrix regulatory factors),<ref name=":0" /> and that through further investigation into all nine, novel therapies and approaches could be proposed on a unique or case-by-case basis should attempts at treating or circumventing complications in any one pathway prove unsuccessful. A number of therapies that target fibroblast activation or the synthesis of extracellular matrix are currently in early testing or are being considered for development.{{citation needed|date=November 2020}}
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A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published by the [[American Thoracic Society]] (ATS) and the [[European Respiratory Society]] (ERS) in 2000 proposed specific major and minor criteria for establishing the diagnosis of IPF.<ref name=Rag2011 /> However, in 2011, new simplified and updated criteria for the diagnosis and management of IPF were published by the ATS, ERS, together with the Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT).<ref name=Rag2011 /> Currently, a diagnosis of IPF requires:
* Exclusion of known causes of [[interstitial lung disease]] (ILD), e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity
* The presence of a typical radiological pattern of [[usual interstitial pneumonia]] (UIP) on [[high-resolution computed tomography]] (HRCT).
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===Medications===
A number of treatments have been investigated in the past for IPF, including [[Interferon beta 1a|interferon gamma-1β]],<ref name=KingAlberaBradford>{{cite journal | vauthors = King TE, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, Noble PW, Sahn SA, Szwarcberg J, Thomeer M, Valeyre D, du Bois RM | display-authors = 6 | title = Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial | journal = Lancet | volume = 374 | issue = 9685 | pages = 222–8 | date = July 2009 | pmid = 19570573 | doi = 10.1016/S0140-6736(09)60551-1 | hdl = 1942/31878 | s2cid = 2432490 | hdl-access = free }}</ref> [[bosentan]],<ref name=KingBrown>{{cite journal | vauthors = King TE, Brown KK, Raghu G, du Bois RM, Lynch DA, Martinez F, Valeyre D, Leconte I, Morganti A, Roux S, Behr J | display-authors = 6 | title = BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis | journal = American Journal of Respiratory and Critical Care Medicine | volume = 184 | issue = 1 | pages = 92–9 | date = July 2011 | pmid = 21474646 | doi = 10.1164/rccm.201011-1874OC | s2cid = 3190634
====Pirfenidone====
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===Lung transplantation===
[[Lung transplantation]] may be suitable for those patients physically eligible to undergo a major transplant operation. In IPF patients, lung transplant has been shown to reduce the risk of death by 75% as compared with patients who remain on the waiting list.<ref name=RussoIribarne>{{cite journal |vauthors=Russo MJ, Iribarne A, Hong KN, Davies RR, Xydas S, Takayama H, Ibrahimiye A, Gelijns AC, Bacchetta MD, D'Ovidio F, Arcasoy S, Sonett JR | title = High lung allocation score is associated with increased morbidity and mortality following transplantation | journal = Chest| volume = 137 | issue = 3 | pages = 651–657 | year = 2010 | pmid = 19820072| pmc = 2832864| doi = 10.1378/chest.09-0319}}</ref> Since the introduction of the [[lung allocation score]] (LAS), which prioritizes transplant candidates based on survival probability, IPF has become the most common indication for lung transplantation in the USA.<ref name=SpagnoloTonelli>{{cite journal |vauthors=Spagnolo P, Tonelli R, Cocconcelli E, Stefani A, Richeldi L | title = Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges | journal = Multidisciplinary Respiratory Medicine| volume = 7 | issue = 1 | page = 42 | year = 2012 | pmid = 23146172| pmc = 3537555| doi = 10.1186/2049-6958-7-42 | doi-access = free }}</ref>
Symptomatic patients with IPF younger than 65 years of age and with a body mass index (BMI) ≤26 kg/m<sup>2</sup> should be referred for lung transplantation, but there are no clear data to guide the precise timing for LTx. Although controversial, the most recent data suggest that bilateral lung transplantation is superior to single lung transplantation in patients with IPF.<ref name=GeorgeArnaoutakis>{{cite journal |vauthors=George TJ, Arnaoutakis GJ, Shah AS | title = Lung transplantation for idiopathic pulmonary fibrosis | journal = The Annals of Thoracic Surgery| volume = 84 | issue = 4 | pages = 1121–1128 | year = 2007 | pmid = 17888957 | doi = 10.1016/j.athoracsur.2007.04.096}}</ref> Five-year survival rates after lung transplantation in IPF are estimated at between 50 and 56%.<ref name=Rag2011 /><ref name=MasonBrizzio>{{cite journal |vauthors=Mason DP, Brizzio ME, Alster JM, McNeill AM, Murthy SC, Budev MM, Mehta AC, Minai OA | title = Lung transplant in idiopathic pulmonary fibrosis | journal = Archives of Surgery| volume = 146 | issue = 10 | pages = 1204–1209 | year = 2011 | pmid = 22006881| doi = 10.1001/archsurg.2011.239|display-authors=etal| doi-access = free }}</ref><ref name=KeatingLevvey>{{cite journal |vauthors=Keating D, Levvey B, Kotsimbos T, Whitford H, Westall G, Williams T, Snell G | title = Lung transplantation in pulmonary fibrosis challenging early outcomes counter balanced by surprisingly good outcomes beyond 15 years | journal = Transplantation Proceedings| volume = 41 | issue = 1 | pages = 289–291 | year = 2009 | pmid = 19249537 | doi = 10.1016/j.transproceed.2008.10.042 }}</ref>
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