Idiopathic pulmonary fibrosis
| Subclass of | idiopathic interstitial pneumonia, pulmonary fibrosis  |
|---|---|
| Health specialty | pulmonology |
| Genetic association | MDGA2, TERT |
| ICD-9-CM | 516.31 |
| NCI Thesaurus ID | C35716, C35715 |
Idiopathic pulmonary fibrosis (IPF), dem formerly know as cryptogenic fibrosing alveolitis,[1] be a rare, progressive illness of de respiratory system, characterized by de thickening den stiffening of lung tissue, wey dey surround de air sacs, wey be associated plus de formation of scar tissue.[2] E be a type of chronic pulmonary fibrosis characterized by a progressive den irreversible decline insyd lung function.[3][4][5]
Symptoms typically dey include gradual onset of shortness of breath den a dry cough.[6] Oda changes fi include feeling tired, den clubbing abnormally large den dome shaped finger den toenails.[6] Complications fi include pulmonary hypertension, heart failure, pneumonia anaa pulmonary embolism.[6]
De cause be unknown, hence de term idiopathic.[7] Risk factors dey include cigarette smoking, gastroesophageal reflux disease, certain viral infections, den genetic predisposition.[6] De underlying mechanism dey involve scarring of de lungs.[6] Diagnosis dey require ruling out oda potential causes.[4] E fi be supported by a high resolution CT scan anaa lung biopsy wich dey show usual interstitial pneumonia.[4] E be a type of interstitial lung disease.[4]
People often dey benefit from pulmonary rehabilitation den supplemental oxygen.[6] Anti-fibrotic medications like pirfenidone anaa nintedanib fi slow de progression of de disease.[7] Lung transplantation sanso fi be an option.[6]
About 5 million people be affected globally.[8] De disease newly dey occur insyd about 12 per 100,000 people per year.[5] Those insyd dema 60s den 70s most be commonly affected.[5] Males be affected more often dan females.[5] Average life expectancy dey follow diagnosis be about four years.[6] Na updated international guidelines dem publish insyd 2022, wich result insyd sam simplification insyd diagnosis den de removal of antacids as a possible adjunct therapy.[9]
References
[edit | edit source]- ↑ "Idiopathic pulmonary fibrosis | Information for the public | Idiopathic pulmonary fibrosis in adults: diagnosis and management | Guidance | NICE". www.nice.org.uk. 12 June 2013. Retrieved 15 June 2024.
- ↑ "Idiopathic Pulmonary Fibrosis". NHLBI, NIH. Retrieved 5 December 2020.
- ↑ Todd, Nevins W.; Luzina, Irina G.; Atamas, Sergei P. (2012). "Molecular and cellular mechanisms of pulmonary fibrosis". Fibrogenesis & Tissue Repair. 5 (1): 11. doi:10.1186/1755-1536-5-11. PMC 3443459. PMID 22824096.
- 1 2 3 4 Raghu G, Collard HR, Egan JJ, et al. (2011). "An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management". American Journal of Respiratory and Critical Care Medicine. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMC 5450933. PMID 21471066.
- 1 2 3 4 Ferri FF (2017). Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1 (in English). Elsevier Health Sciences. p. 691. ISBN 978-0-323-52957-0.
- 1 2 3 4 5 6 7 8 "Idiopathic Pulmonary Fibrosis". NHLBI. Retrieved 21 January 2018.
- 1 2 Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J, et al. (July 2015). "An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline". American Journal of Respiratory and Critical Care Medicine. 192 (2): e3–19. doi:10.1164/rccm.201506-1063ST. PMID 26177183.
- ↑ Meltzer EB, Noble PW (2008). "Idiopathic pulmonary fibrosis". Orphanet Journal of Rare Diseases. 3 (1): 8. doi:10.1186/1750-1172-3-8. PMC 2330030. PMID 18366757.
- ↑ Raghu, Ganesh; Remy-Jardin, Martine; Richeldi, Luca; Thomson, Carey C.; Inoue, Yoshikazu; Johkoh, Takeshi; Kreuter, Michael; Lynch, David A.; Maher, Toby M.; Martinez, Fernando J.; Molina-Molina, Maria; Myers, Jeffrey L.; Nicholson, Andrew G.; Ryerson, Christopher J.; Strek, Mary E. (2022-05-01). "Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline". American Journal of Respiratory and Critical Care Medicine. 205 (9): e18 – e47. doi:10.1164/rccm.202202-0399ST. ISSN 1073-449X. PMC 9851481. PMID 35486072. S2CID 248432749.
External links
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