Interstitial Lung Disease Info

Videos, Podcast, Posts about Pulmonary Fibrosis (lung scarring) and related conditions in an easy-to-understand format. By @scstanel #InterstitialLungDisease

[Stefan Cristian Stanel @scstanel]

Accelerated aging - another perspective on the effects of hypoxia (low oxygen) in chronic respiratory disease.

But oxygen therapy can be given if indicated (levels proven to be low) and generally improves outcomes. Maybe this is one of the reasons why

https://t.co/BZV3Xg71RV

[Interstitial Lung Disease Info @ILDinfo]

I wish we could apply the term "remission" to pulmonary fibrosis. That would be a good aim, to put pulmonary fibrosis into remission.

Why remission? Because when it is progressive, it is a terrible disease. Perhaps we need to be more ambitious with our research objectives. #curePF

[Interstitial Lung Disease Info @ILDinfo]

The diagnosis of the different types of pulmonary fibrosis / ILDs often relies on an ILD multidisciplinary discussion (MDD), where several specialists come together to discuss each case. This group may include the pulmonologist, radiologist with ILD expertise, rheumatologist, specialist nurses, pathologist etc. There may be other specialists joining too depending on the type of case discussed.

This discussion is often needed as cases can be atypical, or only limited information may be available for each patient. Matching the patient's features with the current evidence-based classifications can be difficult, and is often based on probabilities. The final diagnosis is often given a degree of "confidence" following the ILD MDD meeting.

Having a group making the decision regarding the diagnosis and treatment has pros and cons.

Pros may include:
- the experience of each person is brought together as part of the ILD team
- the decision doesn't rest on just one physician
- the risk of making major errors in diagnosis and recommended treatment is reduced
- other members of the medical team can join the meeting and learn to improve their own practice
- other aspects besides the diagnosis can be addressed: need for invasive investigations (e.g. lung biopsy), possible contraindications to certain treatments, social and economic aspects in each case

Cons may include:
- delays in finalizing a diagnosis and treatment plan (as several members of the team need to be in the same meeting at the same time, and each case needs enough allocated time)
- a risk to develop "group-think" especially if the MDD members have all worked together in the same format for many years
- other group dynamics that may affect outcomes (i.e. dominant leader imposing views)
- a preference for standard / established treatment recommendations even in atypical cases (this however may not be a con, as it reduces risk overall)

Balancing these pros and cons, most ILD centres are continuously trying to improve ILD MDDs through standardization (pre-filling case data in a structured way = proformas, specific time and place for a dedicated meeting, no distractions) and incorporating new members into the team who can provide new insights.

The ILD MDD structure is not a perfect, but currently remains the best way to diagnose complex conditions, where clinical information in often not complete.

#pulmonaryfibrosis awareness

[Interstitial Lung Disease Info @ILDinfo]

Asbestosis can sometimes look similar to idiopathic pulmonary fibrosis (IPF) on chest CT scans, but there may be other clues such as pleural plaques (as a marker of asbestos exposure) and a history of significant exposure to asbestos.

Just having an asbestos roof or similar is typically not enough, but rather working with asbestos sheets, brake pads, asbestos insulation, spraying asbestos in various industrial processes etc.

Also, sometimes exposure can be indirect, and I have seen examples such as the wife of an asbestos worker who was washing her husband's clothes for many years.

Quantifying the amount of asbestos exposure is not always easy and requires a careful occupational history.

In many cases, asbestosis-related pulmonary fibrosis remains stable however it can progress in some cases, so would still require monitoring.

#pulmonaryfibrosis awareness
every post counts

[Interstitial Lung Disease Info @ILDinfo]

🫁 Pulmonary fibrosis treatment is more than just antifibrotics (anti "scarring" medication). While these can slow down the decline of progressive pulmonary fibrosis (and IPF), there are other aspects to optimal treatment:

1) Symptom management - May require different medication, such as opioids (morphine, codeine) for breathlessness and cough or expectorants to help with thick sputum. Each ILD patient has a different symptom burden, therefore the medication used may be different.

2) Supportive / palliative care - A holistic approach to the person's care, going beyond usual treatments. Supportive care may involve counselling, psychological and social support, occupational therapy, reducing unnecessary medication and tablet burden, rationalizing the need for invasive tests and procedures etc.

3) Oxygen therapy if required - This is "treatment", not just given for breathlessness. It may help prevent cardiac and other organ system complications.

4) Pulmonary rehabilitation (exercises for the lungs) - These formal programs help to strengthen respiratory muscle function, allowing patients to cope better with their condition.

5) Treating other associated conditions - Pulmonary fibrosis rarely happens in isolation, and many patients have other health conditions that may require treatment optimization. Early referral to other appropriate specialists is very helpful.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Pulmonary fibrosis does not always worsen, but still requires monitoring and observation. This is a more optimistic note, as many patients are very worried about the outcome once diagnosed.

It's important to explain that the disease behavior can be very different from person to person, and survival figures do not always reflect each patient's situation.

Routine follow-up appointments, and repeating lung function tests +/- imaging are helpful to understand each patient's phenotype.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Pulmonary fibrosis needs monitoring as it can sometimes worsen (progressive pulmonary fibrosis - PPF). This is typically done through a combination of routine medical consultations, lung function tests and imaging (chest high-resolution CT scans - HRCT). More details below:

The medical follow-up consultation still has significant value. It gives an opportunity to ask about exercise capacity, coughing, chest infections and other changes to the patient's health. Any changes can be documented and interpreted in the wider context of each person's medical history and other conditions. Breathlessness does not always worsen just because of pulmonary fibrosis, and the clinician can interpret the changes holistically.

Lung function tests give an objective measure of key breathing parameters. For ILD, we typically request spirometry - which measures lung volumes (forced vital capacity - FVC is a key parameter) and DLCO - gas transfer (how quickly we would take up oxygen from the air).

If lung function values drop over time (trend of decline), there may be an indication to repeat the chest CT scan to obtain images of the lungs, and evaluate how the pattern and extent of pulmonary fibrosis has changed. Sometimes, the doctor may request an ultrasound scan of the heart (echocardiogram), blood samples or other tests to see if the worsening in respiratory symptoms is really due to pulmonary fibrosis or other causes.

If there is worsening breathing, worse lung function and progressive pulmonary fibrosis on chest CT scan, the person with pulmonary fibrosis may be eligible for antifibrotic medication (for the progressive pulmonary fibrosis indication), to slow down the decline.

#pulmonaryfibrosis awareness month

[Stefan Cristian Stanel @scstanel]

🫁 In pulmonary fibrosis / ILD, an echocardiogram (heart 🫀 ultrasound scan) is a very useful test for several reasons:

1) Screens for pulmonary hypertension (increased pressure between the right chamber of the heart and the lungs). This complication can significantly worsen breathlessness and outcomes in patients with ILD.

2) Checks if there is heart failure (inability of the heart to pump blood effectively)

3) Evaluates the function of the heart valves.

If the echocardiogram is normal, this reassures patients.

If the echocardiogram is abnormal, then this would:

1) Influence the choice of ILD treatments (if severe abnormalities)

2) Offer an early opportunity to optimize non-ILD therapies (e.g. consider diuretics for congestive heart failure)

3) Facilitate formal pulmonary hypertension evaluation (if detected). Pulmonary hypertension treatments can sometimes help in certain groups of ILD patients.

My personal opinion is that every patient with ILD should have an echocardiogram at least once during their diagnosis and treatment journey. It should be repeated if there is rapid worsening in breathlessness, which is out of keeping with the extent of the pulmonary fibrosis.

#pulmonaryfibrosis awareness month

[Stefan Cristian Stanel @scstanel]

🫁 Not all pulmonary fibrosis is the same. The disease behaviour can be very different from patient to patient. An accurate diagnosis and careful monitoring can help to prioritize interventions for the right people.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

Familial pulmonary fibrosis (FPF) is likely very underdiagnosed

#pulmonaryfibrosis awareness month

[Stefan Cristian Stanel @scstanel]

Pulmonary fibrosis is a great field to implement HealthTech solutions, from wearables to digital stethoscopes - think lung function, lung sounds, sleep patterns etc.

Technological solutions can support early diagnosis, monitoring and prognostication. Lots of interesting research and implementation in progress!

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Pulmonary fibrosis can be familial - this can be both worrying, but also an opportunity for early diagnosis. Up to 1/3 of relatives of patients with idiopathic pulmonary fibrosis (IPF) can have abnormalities on chest CT even when they don't have any breathing problems. Not all will develop an interstitial lung disease, however may require monitoring.

More research into interstitial lung abnormalities (ILAs) is needed to understand how they progress, and who is most at risk. It's a fantastic opportunity for early diagnosis.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Diagnosing pulmonary fibrosis often no longer requires invasive tests.

The field has evolved a lot in the last decade, from requiring lung biopsy for diagnosis to simply looking carefully at the clinical history, chest CT scans, blood and lung function tests. Several other biomarkers and AI tools are currently being developed.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

@hsumit yes, but this is very challenging
However, it is very interesting that pulmonary fibrosis research is very closely related to research into "aging", so may actually lead to paradigm-shifting outcomes across many diseases

[Interstitial Lung Disease Info @ILDinfo]

🫁 More good news regarding idiopathic pulmonary fibrosis (IPF)!
Yet another successful clinical trial after the Fibroneer results of nerandomilast in IPF and progressive pulmonary fibrosis (PPF). 2025 is looking like a great year so far! A lot of progress in the field of pulmonary fibrosis / ILD!

https://t.co/sNBp40nxZq

Fits well with
#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 It is harder to stretch lungs that have scar tissue (pulmonary fibrosis) - the body uses more energy to breathe.

🍲 Eating habits that worked before pulmonary fibrosis was diagnosed are likely no longer optimal. Anti-fibrotic medications can also influence appetite and food absorption. A lot of ILD patients struggle to maintain a normal weight. Keeping an eye on this is very important, as perceived worsening in breathlessness can actually be related to weight loss and deconditioning rather than rapid progression of the pulmonary fibrosis.

Nutritional / dietician advice should be sought early if needed. We may not yet be able to reverse the lung scarring, but a holistic approach to treatment can improve outcomes.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Pulmonary fibrosis is uncommon, but not really that "rare"

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 2 important things about survival in idiopathic pulmonary fibrosis (IPF):

1) The median survival in registries is 3-5 years, however it is very important to know that median = middle number (of a very large range). Some patients can have a faster progression, but many can also live with IPF for a very long time. Disease behavior in individual patients can be very different.

2) IPF typically affects people who are older (70+) and who can have many other health problems. Therefore it is possible that some patients will pass away due to something else (but also having a diagnosis of IPF). It's important to treat holistically, and not just focus on IPF for optimal outcomes.

Idiopathic pulmonary fibrosis (IPF) is not always doom and gloom.

Main thing is to diagnose as early as possible, offer antifibrotic (anti-"scarring") medication if appropriate, and have an overall good management of all IPF health aspects (including other conditions, oxygen assessments, pulmonary rehabilitation, supportive care).

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

🫁 Early signs of pulmonary fibrosis could be an ongoing dry cough, gradually worsening breathlessness, and fine crackles when listening to the lungs with a stethoscope.
The sooner we diagnose pulmonary fibrosis, the better chance we have to change the trajectory of the disease.

#pulmonaryfibrosis awareness month

[Interstitial Lung Disease Info @ILDinfo]

@hsumit Slowing, but this is still a good thing in such a complex condition. There is also a lot of nuance to what constitutes progression

[Interstitial Lung Disease Info @ILDinfo]

Current antifibrotic (anti-"scarring") medication for pulmonary fibrosis includes 2 medications: pirfenidone and nintedanib.
Very likely we'll have a 3rd medication approved soon (end 2025 - 2026): nerandomilast.
These are just some of the medications that can be used, depending on the type of ILD we are dealing with. Treatment options are certainly improving, and it's a very research-active field!

#pulmonaryfibrosis awareness month