What is the risk of worsening (progression) of interstitial lung abnormalities (ILA) in relatives of familial cases of pulmonary fibrosis / ILD? Also, is the current definition for ILA the right one in familial ILD cases?

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I discuss an interesting study done in relatives of patients with ILD / pulmonary fibrosis where abnormalities were found. The authors looked at how these ILAs worsened over ~ 6 years average follow-up. Article here:

https://www.atsjournals.org/doi/10.1164/rccm.202403-0524OChttps://www.atsjournals.org/doi/10.1164/rccm.202403-0524OC Progressive Early Interstitial Lung Abnormalities in Persons at Risk for Familial Pulmonary Fibrosis: A Prospective Cohort StudySalisbury ML, Markin C, Fadely T, Guttentag AR, Humphries SM, Lynch DA, Kropski JA, Blackwell TS. Progressive Early Interstitial Lung Abnormalities in Persons at Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study. Am J Respir Crit Care Med. 2024 Dec 15;210(12):1441-1452. doi: 10.1164/rccm.202403-0524OC. PMID: 39137317; PMCID: PMC11716039.#pulmonaryfibrosis #ild #familial

Interstitial lung abnormalities (ILA) are changes found on chest CT scans that may represent an early form of interstitial lung disease / pulmonary fibrosis. These interstitial abnormalities may be detected before the onset of respiratory symptoms.

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The definition of these changes is based on international expert consensus, but the current radiological reporting is not always standardized. There is a risk that these incidental changes may get worse over time, but the optimal follow-up is not clear (e.g. how often to repeat the chest CT scans). References related to this video:1. Hatabu H, Hunninghake GM, Richeldi L, Brown KK, Wells AU, Remy-Jardin M, Verschakelen J, Nicholson AG, Beasley MB, Christiani DC, San José Estépar R, Seo JB, Johkoh T, Sverzellati N, Ryerson CJ, Graham Barr R, Goo JM, Austin JHM, Powell CA, Lee KS, Inoue Y, Lynch DA. Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society. Lancet Respir Med. 2020 Jul;8(7):726-737. doi: 10.1016/S2213-2600(20)30168-5. PMID: 32649920; PMCID: PMC7970441.2. Hwang J, You S, Lee YJ, Sun JS. Prevalence and progression rate of interstitial lung abnormalities detected on thoracic CT: a systematic review and meta-analysis. Eur Radiol. 2025 Jan;35(1):276-288. doi: 10.1007/s00330-024-10952-9. Epub 2024 Jul 19. PMID: 39026064.3. Grant-Orser A, Min B, Elmrayed S, Podolanczuk AJ, Johannson KA. Prevalence, Risk Factors, and Outcomes of Adult Interstitial Lung Abnormalities: A Systematic Review and Meta-Analysis. Am J Respir Crit Care Med. 2023 Sep 15;208(6):695-708. doi: 10.1164/rccm.202302-0271OC. PMID: 37534937; PMCID: PMC10515575.#pulmonaryfibrosis #ILA #ild Please leave comments below if you want me to cover other topics in ILD.

Screening for pulmonary fibrosis means to test for the presence of signs of lung scarring before symptoms become apparent, to diagnose and treat interstitial lung disease early.

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Some groups of people may be at higher risk of having pulmonary fibrosis: - those diagnosed with a connective tissue disease (autoimmune conditions, such as systemic sclerosis, inflammatory myositis, rheumatoid arthritis etc.) - people with a strong family history of interstitial lung disease / pulmonary fibrosis- those with environmental exposures to dusts, asbestos, mould etc. or those in certain professions (e.g. farmers)Early pulmonary fibrosis is often only detectable on chest CT scans, and deciding who should have such a scan is usually related to the risk factors above. References related to the topic of this episode: 1. Joy GM, Arbiv OA, Wong CK, Lok SD, Adderley NA, Dobosz KM, Johannson KA, Ryerson CJ. Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis. Eur Respir Rev. 2023 Mar 8;32(167):220210. doi: 10.1183/16000617.0210-2022. PMID: 36889782; PMCID: PMC10032591.2. Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A, Froidure A, Galvin L, Griese M, Grutters JC, Molina-Molina M, Poletti V, Prasse A, Renzoni E, van der Smagt J, van Moorsel CHM. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar 16;61(3):2201383. doi: 10.1183/13993003.01383-2022. PMID: 36549714.3. Hunninghake GM, Quesada-Arias LD, Carmichael NE, Martinez Manzano JM, Poli De Frías S, Baumgartner MA, DiGianni L, Gampala-Sagar SN, Leone DA, Gulati S, El-Chemaly S, Goldberg HJ, Putman RK, Hatabu H, Raby BA, Rosas IO. Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis. Am J Respir Crit Care Med. 2020 May 15;201(10):1240-1248. doi: 10.1164/rccm.201908-1571OC. PMID: 32011908; PMCID: PMC7233344.#pulmonaryfibrosis #ild