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?

You can also watch this video on YouTube here: https://youtu.be/DG1HzdCYeLk

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.

Watch video on YouTube here: https://youtu.be/NR2fmlCRZkE

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

Raising a concern about some claims about treatments that may reverse pulmonary fibrosis (lung scarring). Putting this out there just to encourage people to use their common sense and best judgement when choosing health interventions for pulmonary fibrosis and interstitial lung diseases.

You can also watch this episode on YouTube: https://youtu.be/2JRqG2oMt0w

Generally if someone claims that treatment 'X' is the 'best' for pulmonary fibrosis, this could be due to not understanding the topic well enough, not seeing the nuances between different patients or sometimes just trying to sell something... Just be careful and do plenty of research and seek opinions from trained professionals as well before embarking on experimental therapies. #pulmonaryfibrosis #ild

An overview of why a chest CT scan is required in most cases of interstitial lung disease / pulmonary fibrosis. The CT scan is a radiological investigation which offers a 3D picture of the lung tissue, to see where the pulmonary fibrosis (lung scarring) is located, the pattern of fibrosis, and other important lung features which may help your doctor to determine the right treatment, further investigations and tests required, as well as the risk of the fibrosis getting worse over time (progression).

You can also watch this episode on YouTube: https://youtu.be/8aesObW1RFA The simple chest X ray can also pick up pulmonary fibrosis, however this is a 2D image, may miss early disease and cannot describe the pattern of the scarring. The CT scan used to investigate pulmonary fibrosis is called a High Resolution CT scan (HRCT). It is done without i.v. contrast, as the contrast can make some interstitial lung disease changes appear worse than they are. ALWAYS! check with your doctor before having a CT scan, as they are the best person to advise if this investigation is necessary in your case, and they can interpret the findings together with other conditions and symptoms you may have. #pulmonaryfibrosis #ild

Sarcoidosis can lead to severe lung scarring (pulmonary fibrosis). It can also sometimes be associated with pulmonary hypertension. These can significantly impact lung function and cause a drop in oxygen levels, particularly during exertion.

Watch this episode on YouTube: https://youtu.be/qi4D-vLiZJ4

Oxygen saturation levels (SpO2) can be monitored using an oximeter, and values below 90% warrant a check of blood oxygen levels. This can be done through blood gas analysis from the earlobe or an arterial blood sample. If oxygen levels are indeed low, supplemental oxygen therapy may be prescribed to improve oxygen levels, reduce the risk of complications, and potentially enhance exercise tolerance. However, it's worth noting that oxygen therapy may not alleviate all symptoms of breathlessness. Regular follow-ups and additional tests, including imaging and lung function assessments, are crucial to monitor the progression of the disease and manage any potential complications, such as cardiac involvement. Patients experiencing palpitations at rest, or randomly occurring, should inform their doctors, who may recommend further tests like a Holter ECG or cardiac PET to assess heart function and rule out cardiac sarcoidosis. Pulmonary rehabilitation and optimization of other treatments, such as those for other heart and lung conditions, may be beneficial. #sarcoidosis #oxygen #pulmonaryfibrosis

An overview of drug induced interstitial lung disease / pulmonary fibrosis. This video explains how the connection between a certain medication and pulmonary fibrosis is made. You can also watch the video on YouTube here: https://youtu.be/fVRf8Y-hwS4

Several drugs can induce lung reactions and fibrosis, but unless we have a clear timeline it can be very hard to confidently say it was a specific drug that caused the fibrosis.

Remember there may be other causes for pulmonary fibrosis that may need to be investigated.

Look up potential for lung toxicity of different medications here: https://www.pneumotox.com/drug/index/

(a star sign with a higher number in it suggests there is a higher potential of lung toxicity - i.e. number 4 or 5)

#pulmonaryfibrosis #ild #interstitiallungdisease

Overview of interstitial lung abnormalities (ILAs). ILAs represent incidental findings on a chest CT scan, which affect more than 5% of any lung zone (upper, middle or lower) in a person where interstitial lung disease / pulmonary fibrosis was not suspected.

You can also watch this on YouTube here: https://youtu.be/KCrniBdbZic

Interstitial lung abnormalities can be found in 2-9% of patients who have a CT scan (depending on the population studied). There is no clear consensus on how these should be followed up.

The main problem is that in some cases, ILAs may actually represent an early case of pulmonary fibrosis / interstitial lung disease. Therefore there is a risk that the abnormalities may worsen over time.

This is a useful overview of interstitial lung abnormalities: https://pubs.rsna.org/doi/10.1148/radiol.2021204367 #pulmonaryfibrosis #ild