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Fibrosis

MedGen UID:
5179
•Concept ID:
C0016059
•
Pathologic Function
Synonym: Fibroses
SNOMED CT: Fibrosis (112674009); Fibrous repair (112674009); Fibrous thickening (112674009); Fibroplasia (112674009); Fibrous replacement (112674009); Fibrosis (263756000)

Definition

The formation of fibrous tissue. [from NCI]

Professional guidelines

PubMed

Progressive pulmonary fibrosis: an expert group consensus statement.
Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, Mohan A, Renzoni E, Mohan M, Udwadia Z, Shenoy P, Currow D, Devraj A, Jankharia B, Kulshrestha R, Jones S, Ravaglia C, Quadrelli S, Iyer R, Dhooria S, Kolb M, Wells AU
Eur Respir J 2023 Mar;61(3) Epub 2023 Mar 30 doi: 10.1183/13993003.03187-2021. PMID: 36517177Free PMC Article
Treatment of nontuberculous mycobacterial pulmonary disease: an official ATS/ERS/ESCMID/IDSA clinical practice guideline.
Daley CL, Iaccarino JM, Lange C, Cambau E, Wallace RJ Jr, Andrejak C, Böttger EC, Brozek J, Griffith DE, Guglielmetti L, Huitt GA, Knight SL, Leitman P, Marras TK, Olivier KN, Santin M, Stout JE, Tortoli E, van Ingen J, Wagner D, Winthrop KL
Eur Respir J 2020 Jul;56(1) Epub 2020 Jul 7 doi: 10.1183/13993003.00535-2020. PMID: 32636299Free PMC Article
Hepatitis B: Screening, Prevention, Diagnosis, and Treatment.
Wilkins T, Sams R, Carpenter M
Am Fam Physician 2019 Mar 1;99(5):314-323. PMID: 30811163

Recent clinical studies

Etiology

Mitochondria dysfunction and metabolic reprogramming as drivers of idiopathic pulmonary fibrosis.
Bueno M, Calyeca J, Rojas M, Mora AL
Redox Biol 2020 Jun;33:101509. Epub 2020 Mar 19 doi: 10.1016/j.redox.2020.101509. PMID: 32234292Free PMC Article
Atrial fibrillation and cardiac fibrosis.
Sohns C, Marrouche NF
Eur Heart J 2020 Mar 7;41(10):1123-1131. doi: 10.1093/eurheartj/ehz786. PMID: 31713590
Liver Fibrosis Determination.
Lai M, Afdhal NH
Gastroenterol Clin North Am 2019 Jun;48(2):281-289. Epub 2019 Apr 1 doi: 10.1016/j.gtc.2019.02.002. PMID: 31046975
Pulmonary fibrosis, part I: epidemiology, pathogenesis, and diagnosis.
Meyer KC
Expert Rev Respir Med 2017 May;11(5):343-359. Epub 2017 Apr 10 doi: 10.1080/17476348.2017.1312346. PMID: 28345383
Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report.
Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, Lee JS, Maher TM, Wells AU, Antoniou KM, Behr J, Brown KK, Cottin V, Flaherty KR, Fukuoka J, Hansell DM, Johkoh T, Kaminski N, Kim DS, Kolb M, Lynch DA, Myers JL, Raghu G, Richeldi L, Taniguchi H, Martinez FJ
Am J Respir Crit Care Med 2016 Aug 1;194(3):265-75. doi: 10.1164/rccm.201604-0801CI. PMID: 27299520

Diagnosis

Progressive pulmonary fibrosis (PPF).
Duman D
Tuberk Toraks 2022 Dec;70(4):375-381. doi: 10.5578/tt.20229609. PMID: 36537095
Idiopathic pulmonary fibrosis.
Richeldi L, Collard HR, Jones MG
Lancet 2017 May 13;389(10082):1941-1952. Epub 2017 Mar 30 doi: 10.1016/S0140-6736(17)30866-8. PMID: 28365056
Pulmonary fibrosis, part I: epidemiology, pathogenesis, and diagnosis.
Meyer KC
Expert Rev Respir Med 2017 May;11(5):343-359. Epub 2017 Apr 10 doi: 10.1080/17476348.2017.1312346. PMID: 28345383
Idiopathic pulmonary fibrosis.
King TE Jr, Pardo A, Selman M
Lancet 2011 Dec 3;378(9807):1949-61. Epub 2011 Jun 28 doi: 10.1016/S0140-6736(11)60052-4. PMID: 21719092
Retroperitoneal fibrosis.
Vaglio A, Salvarani C, Buzio C
Lancet 2006 Jan 21;367(9506):241-51. doi: 10.1016/S0140-6736(06)68035-5. PMID: 16427494

Therapy

Current Options and Future Directions for NAFLD and NASH Treatment.
Zhang C, Yang M
Int J Mol Sci 2021 Jul 15;22(14) doi: 10.3390/ijms22147571. PMID: 34299189Free PMC Article
Progression of fibrosing interstitial lung disease.
Wong AW, Ryerson CJ, Guler SA
Respir Res 2020 Jan 29;21(1):32. doi: 10.1186/s12931-020-1296-3. PMID: 31996266Free PMC Article
Respiratory muscle training in children and adults with neuromuscular disease.
Silva IS, Pedrosa R, Azevedo IG, Forbes AM, Fregonezi GA, Dourado Junior ME, Lima SR, Ferreira GM
Cochrane Database Syst Rev 2019 Sep 5;9(9):CD011711. doi: 10.1002/14651858.CD011711.pub2. PMID: 31487757Free PMC Article
Physical exercise training for cystic fibrosis.
Radtke T, Nevitt SJ, Hebestreit H, Kriemler S
Cochrane Database Syst Rev 2017 Nov 1;11(11):CD002768. doi: 10.1002/14651858.CD002768.pub4. PMID: 29090734Free PMC Article
Pathobiology of liver fibrosis: a translational success story.
Lee YA, Wallace MC, Friedman SL
Gut 2015 May;64(5):830-41. Epub 2015 Feb 13 doi: 10.1136/gutjnl-2014-306842. PMID: 25681399Free PMC Article

Prognosis

Nintedanib with Add-on Pirfenidone in Idiopathic Pulmonary Fibrosis. Results of the INJOURNEY Trial.
Vancheri C, Kreuter M, Richeldi L, Ryerson CJ, Valeyre D, Grutters JC, Wiebe S, Stansen W, Quaresma M, Stowasser S, Wuyts WA; INJOURNEY Trial Investigators
Am J Respir Crit Care Med 2018 Feb 1;197(3):356-363. doi: 10.1164/rccm.201706-1301OC. PMID: 28889759
Background and Epidemiology.
Sanders DB, Fink AK
Pediatr Clin North Am 2016 Aug;63(4):567-84. doi: 10.1016/j.pcl.2016.04.001. PMID: 27469176Free PMC Article
Idiopathic pulmonary fibrosis: moving forward.
Richeldi L
BMC Med 2015 Sep 24;13:231. doi: 10.1186/s12916-015-0481-6. PMID: 26399729Free PMC Article
Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review.
Hutchinson J, Fogarty A, Hubbard R, McKeever T
Eur Respir J 2015 Sep;46(3):795-806. Epub 2015 May 14 doi: 10.1183/09031936.00185114. PMID: 25976683
Pyogenic lung infections.
Mwandumba HC, Beeching NJ
Curr Opin Pulm Med 1999 May;5(3):151-6. doi: 10.1097/00063198-199905000-00005. PMID: 10228739

Clinical prediction guides

Development and Validation of a Score for Fibrotic Nonalcoholic Steatohepatitis.
Tavaglione F, Jamialahmadi O, De Vincentis A, Qadri S, Mowlaei ME, Mancina RM, Ciociola E, Carotti S, Perrone G, Bruni V, Gallo IF, Tuccinardi D, Bianco C, Prati D, Manfrini S, Pozzilli P, Picardi A, Caricato M, Yki-Järvinen H, Valenti L, Vespasiani-Gentilucci U, Romeo S
Clin Gastroenterol Hepatol 2023 Jun;21(6):1523-1532.e1. Epub 2022 Apr 11 doi: 10.1016/j.cgh.2022.03.044. PMID: 35421583
Long-term outcomes and predictive ability of non-invasive scoring systems in patients with non-alcoholic fatty liver disease.
Younes R, Caviglia GP, Govaere O, Rosso C, Armandi A, Sanavia T, Pennisi G, Liguori A, Francione P, Gallego-Durán R, Ampuero J, Garcia Blanco MJ, Aller R, Tiniakos D, Burt A, David E, Vecchio FM, Maggioni M, Cabibi D, Pareja MJ, Zaki MYW, Grieco A, Fracanzani AL, Valenti L, Miele L, Fariselli P, Petta S, Romero-Gomez M, Anstee QM, Bugianesi E
J Hepatol 2021 Oct;75(4):786-794. Epub 2021 Jun 4 doi: 10.1016/j.jhep.2021.05.008. PMID: 34090928
Cardiac Magnetic Resonance in Nonischemic Cardiomyopathies.
Yang EY, Shah DJ
Methodist Debakey Cardiovasc J 2020 Apr-Jun;16(2):97-105. doi: 10.14797/mdcj-16-2-97. PMID: 32670469Free PMC Article
Histopathology of nonalcoholic fatty liver disease/nonalcoholic steatohepatitis.
Takahashi Y, Fukusato T
World J Gastroenterol 2014 Nov 14;20(42):15539-48. doi: 10.3748/wjg.v20.i42.15539. PMID: 25400438Free PMC Article
Design and validation of a histological scoring system for nonalcoholic fatty liver disease.
Kleiner DE, Brunt EM, Van Natta M, Behling C, Contos MJ, Cummings OW, Ferrell LD, Liu YC, Torbenson MS, Unalp-Arida A, Yeh M, McCullough AJ, Sanyal AJ; Nonalcoholic Steatohepatitis Clinical Research Network
Hepatology 2005 Jun;41(6):1313-21. doi: 10.1002/hep.20701. PMID: 15915461

Recent systematic reviews

Improving nutrition in cystic fibrosis: A systematic literature review.
Mielus M, Sands D, Woynarowski M
Nutrition 2022 Oct;102:111725. Epub 2022 May 6 doi: 10.1016/j.nut.2022.111725. PMID: 35816813
Efficacy of antifibrotic drugs, nintedanib and pirfenidone, in treatment of progressive pulmonary fibrosis in both idiopathic pulmonary fibrosis (IPF) and non-IPF: a systematic review and meta-analysis.
Finnerty JP, Ponnuswamy A, Dutta P, Abdelaziz A, Kamil H
BMC Pulm Med 2021 Dec 11;21(1):411. doi: 10.1186/s12890-021-01783-1. PMID: 34895203Free PMC Article
Physical exercise training for cystic fibrosis.
Radtke T, Nevitt SJ, Hebestreit H, Kriemler S
Cochrane Database Syst Rev 2017 Nov 1;11(11):CD002768. doi: 10.1002/14651858.CD002768.pub4. PMID: 29090734Free PMC Article
Increased risk of mortality by fibrosis stage in nonalcoholic fatty liver disease: Systematic review and meta-analysis.
Dulai PS, Singh S, Patel J, Soni M, Prokop LJ, Younossi Z, Sebastiani G, Ekstedt M, Hagstrom H, Nasr P, Stal P, Wong VW, Kechagias S, Hultcrantz R, Loomba R
Hepatology 2017 May;65(5):1557-1565. Epub 2017 Mar 31 doi: 10.1002/hep.29085. PMID: 28130788Free PMC Article
Fibrosis progression in nonalcoholic fatty liver vs nonalcoholic steatohepatitis: a systematic review and meta-analysis of paired-biopsy studies.
Singh S, Allen AM, Wang Z, Prokop LJ, Murad MH, Loomba R
Clin Gastroenterol Hepatol 2015 Apr;13(4):643-54.e1-9; quiz e39-40. Epub 2014 Apr 24 doi: 10.1016/j.cgh.2014.04.014. PMID: 24768810Free PMC Article

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