No cure exists and treatment options are limited.[1] Treatment is directed toward improving symptoms and may include oxygen therapy and pulmonary rehabilitation.[1][4] Certain medications may slow the scarring.[4]Lung transplantation may be an option.[3] At least 5 million people are affected globally.[5] Life expectancy is generally less than five years.[3]
Pulmonary fibrosis is suggested by a history of progressive shortness of breath (dyspnea) with exertion. Sometimes fine inspiratory crackles can be heard at the lung bases on auscultation. A chest X-ray may not be abnormal, but high-resolution CT will often show abnormalities.[3]
Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections, and bacterial infections such as tuberculosis that may cause fibrotic changes in the lungs' upper or lower lobes and other microscopic lung injuries. But pulmonary fibrosis can also appear without any known cause. In that case, it is termed "idiopathic".[7] Most idiopathic cases are diagnosed as idiopathic pulmonary fibrosis. This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, a growing body of evidence points to a genetic predisposition in a subset of patients. For example, a mutation in surfactant protein C (SP-C) has been found in some families with a history of pulmonary fibrosis.[8]Autosomal dominant mutations in the TERC or TERT genes, which encode telomerase, have been identified in about 15% of pulmonary fibrosis patients.[9]
Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include:[3][8]
Inhalation of environmental and occupational pollutants, such as metals[10] in asbestosis, silicosis, and exposure to certain gases. Coal miners, ship workers and sand blasters, among others, are at higher risk.[7]
Hypersensitivity pneumonitis, most often resulting from inhaling dust contaminated with bacterial, fungal, or animal products
Cigarette smoking can increase the risk or make the illness worse.[7] Smoking is a known cause of some types of lung fibrosis, such as smoking-related interstitial fibrosis (SRIF).[11]
Pulmonary fibrosis involves a gradual replacement of normal lung tissue with fibrotic tissue. Such scar tissue causes an irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease.[14] Pulmonary fibrosis is perpetuated by aberrant wound healing, rather than chronic inflammation.[15] It is the main cause of restrictive lung disease that is intrinsic to the lung parenchyma. In contrast, quadriplegia[16] and kyphosis[17] are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis.
TGF-β is a cytokine that plays a critical role in the regulation of extracellular matrix (ECM) production and cellular differentiation.[26] It is a potent stimulator of fibrosis, and increased TGF-β signaling is associated with the development of fibrosis in various organs.
CTGF is a matricellular protein involved in ECM production and remodeling.[26] It is up-regulated in response to TGF-β and has been implicated in the development of pulmonary fibrosis.[18]
EGFR is a transmembrane receptor that plays a role in cellular proliferation, differentiation, and survival. Dysregulated EGFR signaling has been implicated in the development of pulmonary fibrosis, and drugs that target EGFR have been shown to have therapeutic potential in the treatment of the disease.[20]
IL-13 is a cytokine involved in regulating immune responses.[21] It has been shown to promote fibrosis in the lungs by stimulating the production of ECM proteins and the recruitment of fibroblasts to sites of tissue injury.
PDGF is a cytokine that plays a key role in the regulation of cell proliferation and migration.[22] It is involved in the recruitment of fibroblasts to sites of tissue injury in the lungs, and increased PDGF signaling is associated with the development and progression of pulmonary fibrosis.
Wnt/β-catenin signaling plays a critical role in tissue repair and regeneration, and dysregulated Wnt/β-catenin signaling has been implicated in the development of pulmonary fibrosis.[23]
Diagnosis
The diagnosis can be confirmed by lung biopsy.[3] A video-assisted thoracoscopic surgery (VATS) under general anesthesia may be needed to obtain enough tissue to make an accurate diagnosis. This kind of biopsy involves placement of several tubes through the chest wall, one of which is used to cut off a piece of lung for evaluation. The removed tissue is examined histopathologically by microscopy to confirm the presence and pattern of fibrosis as well as other features that may indicate a specific cause, such as specific types of mineral dust or possible response to therapy, e.g. a pattern of so-called non-specific interstitial fibrosis.
Misdiagnosis is common because, while pulmonary fibrosis is not rare, each type is uncommon and evaluation of patients with these diseases is complex and requires a multidisciplinary approach. Terminology has been standardized but difficulties still exist in their application. Even experts may disagree on the classification of some cases.[27]
On spirometry, as a restrictive lung disease, both the FEV1 (forced expiratory volume in 1 second) and FVC (forced vital capacity) are reduced so the FEV1/FVC ratio is normal or even increased, in contrast to obstructive lung disease, where this ratio is reduced. The values for residual volume and total lung capacity are generally decreased in restrictive lung disease.[28]
Treatment
Pulmonary fibrosis creates scar tissue. The scarring is permanent once it has developed.[29] Slowing the progression and prevention depends on the underlying cause:
Treatment options for idiopathic pulmonary fibrosis are very limited, since no current treatment has stopped the progression of the disease. Because of this, there is no evidence that any medication can significantly help this condition, despite ongoing research trials. Lung transplantation is the only therapeutic option available in severe cases. A lung transplant can improve the patient's quality of life.[30]
Immunosuppressive drugs can also be considered. These are sometimes prescribed to slow the processes that lead to fibrosis. Some types of lung fibrosis respond to corticosteroids, such as prednisone.[29]
Oxygen therapy is also an option. The patient may choose how much oxygen to use. The use of oxygen doesn't repair the lung damage, but can:
make breathing and exercise easier;
prevent or lessen complication from low blood oxygen levels;
The immune system is thought to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immunosuppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment vary. Those whose conditions improve with immunosuppressive treatment probably do not have idiopathic pulmonary fibrosis, for idiopathic pulmonary fibrosis has no significant treatment or cure. [30]
Two pharmacological agents intended to prevent scarring in mild idiopathic fibrosis are pirfenidone, which reduced reductions in the 1-year rate of decline in FVC and reduced the decline in distances on the 6-minute walk test, but had no effect on respiratory symptoms,[31] and is nintedanib, which acts as an antifibrotic, mediated through the inhibition of a variety of tyrosine kinase receptors (including platelet-derived growth factor, fibroblast growth factor, and vascular endothelial growth factor).[32] A randomized clinical trial showed it reduced lung-function decline and acute exacerbations.[33]
Hypersensitivity pneumonitis, a less severe form of pulmonary fibrosis, is prevented from becoming aggravated by avoiding contact with the causative material.
Globally, the prevalence and incidence of pulmonary fibrosis has been studied in the United States, Norway, Czech Republic, Greece, United Kingdom, Finland, and Turkey, with only two studies in Japan and Taiwan. But most of these studies were of people already diagnosed with pulmonary fibrosis, which lowers the diagnosis sensitivity, so that the prevalence and incidence has ranged from 0.7 per 100,000 in Taiwan to 63.0 per 100,000 in the U.S., and the published incidence has ranged from 0.6 per 100,000 person years to 17.4 per 100,000 person years.[34]
The mean age of all pulmonary fibrosis patients is between 65 and 70 years, making age a criterion of its own. Aging respiratory systems are much more vulnerable to fibrosis and stem cell depletion.
Based on these rates, pulmonary fibrosis prevalence in the U.S. could range from more than 29,000 to almost 132,000, based on the population in 2000 that was 18 years or older. The actual number may be significantly higher due to misdiagnosis. Typically, patients are in their forties and fifties when diagnosed, while the incidence of idiopathic pulmonary fibrosis increases dramatically after age 50. But loss of pulmonary function is commonly ascribed to old age, heart disease, or more common lung diseases.[39]
Since the COVID-19 pandemic, deaths of people with pulmonary fibrosis increased due to the rapid loss of pulmonary function. The consequences of COVID-19 include a large cohort of patients with both fibrosis and progressive lung impairment. Long-term follow-up studies are showing long-term impairment of lung function and radiographic abnormalities suggestive of pulmonary fibrosis for patients with lung comorbidities.[40]
The most common long-term consequence in COVID-19 patients is pulmonary fibrosis. The biggest concerns about pulmonary fibrosis and the increase of respiratory follow-up after COVID-19 are expected to be solved in the near future. Older age with decreased lung function and/or preexisting comorbidities, such as diabetes, cardiovascular disease, hypertension, and obesity increase the risk of developing fibrotic lung alterations in COVID-19 survivors with lower exercise tolerance. According to one study, 40% of COVID-19 patients develop a form of fibrosis of the lungs, and 20% of those are severe.[41]
^Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and Prevalence of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2006;174:810-6.
^Fernandez Perez ER, Daniels CE, Schroeder DR, St Sauver J, Hartman TE, Bartholmai BJ, Yi ES, Ryu JH. Incidence, Prevalence, and Clinical Course of Idiopathic Pulmonary Fibrosis: A Population-Based Study. Chest. Jan 2010;137:129-37.
^Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The Epidemiology of Interstitial Lung Diseases. Am J Respir Crit Care Med. Oct 1994;150(4):967-72. cited by Michaelson JE, Aguayo SM, Roman J. Idiopathic Pulmonary Fibrosis: A Practical Approach for Diagnosis and Management. Chest. Sept 2000;118:788-94.
Jawaharlal Nehru, Bhulabhai Desai dan Babu Rajendra Prasad (tengah) di Sesi AICC, April 1939 Bhulabhai Desai (13 Oktober 1877 – 6 Mei 1946) adalah seorang aktivis kemerdekaan dan pengacara India. Ia dikenal karena membela tiga prajurit Tentara Nasional India yang dituduh melakukan penyiksaan pada masa Perang Dunia II, dan berupaya menegosiasikan perjanjian pembagian kekuasaan rahasia dengan Liaquat Ali Khan dari Liga Muslim. Referensi Rajmohan Gandhi, Patel: A Life (1992) Motilal…
Artikel ini sebatang kara, artinya tidak ada artikel lain yang memiliki pranala balik ke halaman ini.Bantulah menambah pranala ke artikel ini dari artikel yang berhubungan atau coba peralatan pencari pranala.Tag ini diberikan pada Maret 2016. Jota Aviation IATA ICAO Kode panggil -[1] ENZ ENZO Didirikan2009Armada7Perusahaan indukJota GroupKantor pusatSouthend, Britania RayaSitus web[1] Jota Aviation adalah sebuah maskapai penerbangan sewaan yang berbasis di Bandar Udara London Southend, B…
هذه المقالة يتيمة إذ تصل إليها مقالات أخرى قليلة جدًا. فضلًا، ساعد بإضافة وصلة إليها في مقالات متعلقة بها. (نوفمبر 2019) التصميم الهندسي للطرق: هو فرع من هندسة الطرق يُعنى بوضع العناصر للطريق وفقًا للمعايير. يهدف التصميم الهندسي بشكل أساسي إلى تحسين الكفاءة والسلامة مع تقليل ال…
Puluhan film tuturan dari seri Detektif Conan telah dirilis sejak 1997 di Jepang dan beberapa negara. Film-film tersebut dirilis setiap bulan April sejak tahun 1997, tidak termasuk tahun 2020. Setiap film memiliki alur cerita tesendiri dan bukan merupakan adaptasi dari cerita manga. Di antara film-film ini, hanya 23 film pertama yang pernah ditayangkan di Indonesia melalui Indosiar dan Net.. Sejak 2016, film tuturan Detektif Conan mulai ditayangkan di bioskop Indonesia, melalui jaringan bioskop …
Artikel ini membahas mengenai narkotika, psikotropika, dan zat adiktif lainnya. Informasi mengenai zat dan obat-obatan terlarang hanya dimuat demi kepentingan ilmu pengetahuan. Kepemilikan dan pengedaran narkoba adalah tindakan melanggar hukum di berbagai negara. Baca: penyangkalan umum lihat pula: nasihat untuk orang tua. Untuk kegunaan lain, lihat Ganja (disambiguasi). Untuk tumbuhan, lihat Cannabis. Ganja atau mariyuana adalah psikotropika mengandung tetrahidrokanabinol sebagai senyawa kimia …
Pikiran RakyatDari Rakyat - Oleh Rakyat - Untuk RakyatTipeSurat kabar harianFormatLembar lebarPemilikGrup Pikiran RakyatPenerbitPikiran Rakyat BandungDidirikan24 Maret 1966; 58 tahun lalu (1966-03-24)BahasaIndonesiaPusatBandung, IndonesiaSitus webwww.pikiran-rakyat.comArsip daring gratisePaper Pikiran Rakyat Pikiran Rakyat adalah surat kabar harian yang terbit di Bandung, Indonesia. Peredarannya meliputi Jawa Barat dan Banten. Sejarah Pada Bulan Januari 1966, di Kota Bandung terdapat sejuml…
Peta lokasi Madagaskar di Afrika. Rencana Madagaskar (Jerman: Madagaskarplancode: de is deprecated atau Madagaskar-Plan) adalah usulan pemerintah Jerman Nazi untuk memindahkan populasi Yahudi Eropa ke pulau Madagaskar.[1] Franz Rademacher, kepala Departemen Yahudi di Kementerian Luar Negeri Nazi, mengungkapkan gagasan ini pada Juni 1940, beberapa saat sebelum kekalahan Prancis dalam Pertempuran Prancis. Usulan ini meminta agar Madagaskar, yang merupakan koloni Prancis saat itu, diserahka…
For the surrounding metropolitan area, see Northwest Arkansas. For the city of a similar name, see Benton, Arkansas. City in Arkansas, United StatesBentonville, ArkansasCityDowntown Bentonville at nightCrystal Bridges Museum of American ArtBenton County Courthouse FlagInteractive map of BentonvilleBentonvilleShow map of ArkansasBentonvilleShow map of the United StatesCoordinates: 36°21′10″N 94°13′52″W / 36.35278°N 94.23111°W / 36.35278; -94.23111CountryUnited …
Jembatan pelengkung Pont Alexandre III di kota Paris dan Jembatan Trinity di kota St Petersburg tetap menjadi dua lambang atas bersekutunya Prancis dan Rusia. Aliansi Prancis-Rusia terbentuk karena sebuah perjanjian pada tahun 1891 sampai 1893 yang terus berlangsung hingga tahun 1917. Penyebab adanya kebijakan luar negeri umum dan kepentingan militer strategis bersama dari Prancis dan Rusia ini berawal dari menguatnya zaman kekaisaran Jerman, diciptakannya Aliansi Tiga pada tahun 1882 dan membur…
You can help expand this article with text translated from the corresponding article in German. (February 2022) Click [show] for important translation instructions. View a machine-translated version of the German article. Machine translation, like DeepL or Google Translate, is a useful starting point for translations, but translators must revise errors as necessary and confirm that the translation is accurate, rather than simply copy-pasting machine-translated text into the English Wikipedi…
Belgian research and documentation center Jesuit European Social CentreAbbreviationJESCEstablished1956; 68 years ago (1956)LocationRue du CornetBrussels, BelgiumCoordinates48°35′13.19″N 7°44′41.88″E / 48.5869972°N 7.7449667°E / 48.5869972; 7.7449667AffiliationsJesuit, CatholicWebsitejesc.euFormerly calledCatholic Office of Information and Initiative for Europe (OCIPE) Jesuit European Social Centre (JESC), formerly known as Catholic Office of …
Egyptian footballer Ali El-Hassani El-Hassani with Egypt in the 1928 Olympic GamesPersonal informationDate of birth January 1897Place of birth Cairo, EgyptDate of death 1976 (aged 78–79)[1]Senior career*Years Team Apps (Gls)1914-1923 Zamalek 1924-1927 Al Ahly 1927-1929 Zamalek 1929-1931 Al Ahly 1931-1934 Zamalek 1934-1936 El Sekka El Hadid International career1920-1928 Egypt *Club domestic league appearances and goals Ali El-Hassani (January 1897 – 1976) was an Egyptian …
Virtual International Authority File (VIAF)sito webVIAF: Screenshot (2012) URLviaf.org/ Tipo di sitoMotore di ricerca, catalogo pubblico on-line LinguaInglese Registrazioneno Commercialeno ProprietarioOnline Computer Library Center Creato daProgetto congiunto di biblioteche nazionali Lancio2003 Stato attualeattivo Modifica dati su Wikidata · Manuale Il Virtual International Authority File (acronimo: VIAF) è un progetto internazionale gestito dall'Online Computer Library Center (OCLC) che …
New York City Subway service New York City Subway serviceQueens Boulevard Express/ Eighth Avenue LocalQueens bound E train of R160As enters 42nd Street–Port Authority Bus TerminalNote: This map represents normal service. Dashed line shows late night only serviceDashed pink line shows weekday rush hour, midday and early evening service to 179th StreetNorthern endJamaica Center–Parsons/Archer (all times)Jamaica–179th Street (weekday rush hour and early evenings)Southern endWorld Tr…
2011 single by Young Jeezy featuring Lil WayneBallin'Single by Young Jeezy featuring Lil Waynefrom the album Thug Motivation 103: Hustlerz Ambition ReleasedMay 17, 2011Recorded2011GenreTrapLength4:43LabelDef JamSongwriter(s)Jay Jenkins, Antoine Kearney, D. Ellerbee, Dwayne CarterProducer(s)Lil LodyYoung Jeezy singles chronology Lose My Mind (2010) Ballin' (2011) F.A.M.E. (2011) Lil Wayne singles chronology 9 Piece(2011) Ballin'(2011) I'm on One(2011) Ballin' is a song by …
Family of DNA sequence found in prokaryotic organisms This article is about the prokaryotic antiviral system. For the use in editing genes, see CRISPR gene editing. Cascade (CRISPR-associated complex for antiviral defense)CRISPR Cascade protein (cyan) bound to CRISPR RNA (green) and phage DNA (red)[1]IdentifiersOrganismEscherichia coliSymbolCRISPREntrez947229PDB4QYZRefSeq (Prot)NP_417241.1UniProtP38036Search forStructuresSwiss-modelDomainsInterPro Part of a series onCRISPR Genome …
Political subdivision in Malaysia This article relies largely or entirely on a single source. Relevant discussion may be found on the talk page. Please help improve this article by introducing citations to additional sources.Find sources: Kijal state constituency – news · newspapers · books · scholar · JSTOR (April 2020) Kijal Terengganu constituencyState constituencyLegislatureTerengganu State Legislative AssemblyMLA Razali IdrisPN…
Anna Smashnovaאנה סמשנובה Anna Smashnova al servizio Nazionalità Israele Altezza 157 cm Peso 53 kg Tennis Termine carriera luglio 2007 Carriera Singolare1 Vittorie/sconfitte 401–304 Titoli vinti 12 WTA, 7 ITF Miglior ranking 15º (3 febbraio 2003) Risultati nei tornei del Grande Slam Australian Open 3T (1995, 2003, 2005) Roland Garros 4T (1995, 1998) Wimbledon 3T (2000) US Open 3T (1994) Altri tornei Giochi olimpici 1T (2004) Doppio1 Vittorie/scon…
У этого термина существуют и другие значения, см. Чайки (значения). Чайки Доминиканская чайкаЗападная чайкаКалифорнийская чайкаМорская чайка Научная классификация Домен:ЭукариотыЦарство:ЖивотныеПодцарство:ЭуметазоиБез ранга:Двусторонне-симметричныеБез ранга:Вторичн…
For Ronan Keating's group recordings, see Boyzone. Ronan Keating discographyKeating performing in 2012Studio albums12Live albums4Compilation albums1Music videos28Singles32 Irish pop singer Ronan Keating has released twelve studio albums, one compilation album and thirty-two singles. His solo career started in 1999 and has spawned nine albums. He gained worldwide attention when his single When You Say Nothing at All was featured in the film Notting Hill and peaked at number one in several countri…