About this webinar
What is pulmonary hypertension associated with interstitial lung disease (PH-ILD), and how can we improve care for people living with it? In this joint webinar from the European Pulmonary Fibrosis Federation and the Alliance for Pulmonary Hypertension, clinical and patient perspectives come together to explore earlier recognition and better support. Dr. Esther Nossent from Amsterdam UMC explains how PH-ILD develops, its effects on the lungs and heart, and why it can be difficult to recognise when symptoms overlap with those of existing lung disease. She discusses the investigations used to assess suspected PH-ILD, approaches to individualised treatment and supportive care, and the role of research in developing further treatment options. Chaired by Louise Bouman, the panel discussion with Dr Nossent and Liam Galvin explores how closer collaboration between ILD and pulmonary hypertension specialists can improve the patient journey. Topics include clear referral pathways, communication about tests and next steps, and the role of patient organisations in providing reliable information and peer support. The speakers also address psychological support and the needs of families and carers, emphasising that care and research should reflect what matters to patients in daily life. This webinar is for people living with PH-ILD, their families and carers, patient organisations, and healthcare professionals seeking a better understanding of the condition.
Transcript
Louise Bouman
Hello everyone, and welcome to our webinar, Together for PH-ILD: Strengthening Awareness, Care and Patient Support. My name is Louise, and I am joining you on behalf of the Alliance for Pulmonary Hypertension, where I serve as Vice President. I will be your chair this evening.
I am very pleased that we can bring you this webinar through a collaboration between the European Pulmonary Fibrosis Federation and the Alliance for Pulmonary Hypertension. Both organisations recognise the importance of raising awareness of pulmonary hypertension associated with interstitial lung disease, or PH-ILD, and working together to improve care, information and support for people living with this condition.
We have an excellent programme ahead of us. First, I am delighted to introduce Dr Esther Nossent from Amsterdam UMC in the Netherlands, who will give us an introduction to PH-ILD. Esther is a pulmonologist with expertise in both pulmonary hypertension and ILD. She will explain the basics and help us build a shared understanding of what PH-ILD means and why it matters.
Following Esther’s presentation, we will move into a panel discussion. Esther and Liam Galvin, a board member of the European Pulmonary Fibrosis Federation, will join me to explore how we can strengthen collaboration to improve awareness, care and support for people living with PH-ILD.
We will also leave time for questions from our audience. Please feel free to submit your questions at any time using the chat function. You do not need to wait until the Q&A session: we will collect your questions and address as many as possible at the end of the webinar. With that, I would like to hand over to Esther. Esther, the floor is yours.
Dr Esther Nossent
Thank you, Louise, and thank you, Louise and Liam, for inviting me to participate in this important webinar. Good evening to everyone at home. I will start by sharing my screen and checking that everyone can see it.
I am going to talk about pulmonary hypertension associated with interstitial lung disease. On this first slide, you can see CT images from one of my patients. Many of you will have had CT scans yourselves. These images show interstitial lung disease in both lungs, a large pulmonary artery and an enlarged right side of the heart, which lies between the lungs. Tonight, I will explain why I am starting with these pictures and what it means to develop pulmonary hypertension associated with ILD.
We often shorten the name to PH-ILD. Louise and Liam asked me to explain the basics, so I will start with what PH-ILD is and how it develops. These are my conflicts of interest. I would then like to begin with a study about the voices of European patients living with PH-ILD.
Healthcare professionals and researchers carried out a small survey and study, asking patients and carers about the impact of PH-ILD on daily life and their experiences during diagnosis and treatment. A few things struck me when I read it. PH-ILD has a profound impact on daily life, including mobility and exercise capacity. The diagnostic journey can also be challenging, which is important to keep in mind throughout this presentation.
For people living with PH-ILD, successful care is not only about improving clinical measurements or test results. It is about preserving independence, mobility and, especially, quality of life. That is something we will focus on tonight.
First, what is interstitial lung disease, and what is pulmonary fibrosis? There are many different forms of ILD. Examples include idiopathic pulmonary fibrosis, or IPF; hypersensitivity pneumonitis; sarcoidosis; and ILD associated with rheumatoid arthritis or scleroderma. Those are only a few examples.
Here are CT scans from patients with different forms of ILD. You can see that the four scans look quite different; you do not have to be a pulmonologist to recognise that. The different types have different causes, require different diagnostic approaches and need different treatments. There is no single treatment for every person with ILD. That is important to remember.
Some forms of ILD can lead to pulmonary fibrosis. People often mix up the terms “interstitial lung disease” and “pulmonary fibrosis”. Pulmonary fibrosis means scarring of the lung tissue. When this happens, lung tissue is damaged and destroyed. As with scarring on the skin, the scarring also makes the lungs stiffer.
This CT scan shows a patient with pulmonary fibrosis. You can see areas that look like black holes, compared with areas of normal lung tissue. There is destruction of the lung tissue, which we call the lung parenchyma. When fibrosis makes the lungs stiffer, breathing takes more work because the lungs cannot move as easily. With less functioning lung tissue, it is also more difficult to take up oxygen.
Turning to pulmonary hypertension, this means high blood pressure in the blood vessels of the lungs. It is different from the blood pressure measured in your arm. Pulmonary hypertension describes increased pressure in these vessels; the measurement alone does not tell us the underlying disease or cause.
Some clinical investigations can suggest that pulmonary hypertension may be present, such as an ultrasound of the heart. We can also measure pressures in the lungs and heart through right heart catheterisation, which I will discuss later. If the pressure is high, we call that pulmonary hypertension, but we still need to understand what is causing it.
Let me explain how the circulation normally works. Following my pointer on the diagram, blood returns from the body to the right side of the heart, entering the right atrium and then the right ventricle, or right chamber. From there, it travels to the lungs. In the lungs, the blood takes up oxygen and releases carbon dioxide.
The oxygen-rich blood then returns to the left side of the heart, entering the left atrium and then the left ventricle. The left ventricle pumps it around the body again. The important point is that the right ventricle pumps blood into the lung blood vessels so that the blood can pick up oxygen.
Normally, this is an easy process. The lung blood vessels are wide and open, and there are many of them. Resistance is low, so blood flows through easily and can take up oxygen. It is a low-pressure system: the right side of the heart does not have to work very hard to push blood through the lungs.
What happens in pulmonary hypertension? I often compare it with a garden hose. On the left of this image, the hose is wide, so water flows through easily with low resistance and little pressure. If the hose narrows, resistance increases, and higher pressure is needed to push the water through.
A similar process happens in pulmonary hypertension. Different mechanisms can reduce the number of blood vessels, block them or cause them to narrow. Narrowed vessels create higher resistance, so more pressure is needed to move blood through the lungs. That is why the pressure rises.
The right side of the heart then has to pump harder. Sometimes it is almost as though it has to run a marathon to get blood through the lungs. Initially, the right ventricle may adapt, but if the pressure becomes very high, right heart failure can develop. The heart can no longer cope with the pressure it is working against.
Pulmonary hypertension therefore involves high pressure in the lung blood vessels, but the consequences for the right side of the heart are also important. It has to work harder, and that can lead to right heart failure. So what happens specifically in PH-ILD?
This is the CT scan I showed earlier, with fibrosis and destruction of lung tissue. You can imagine that when lung tissue is destroyed, some of its blood vessels are lost as well. The fibrosis and the disease process can also affect the remaining vessels, causing them to narrow.
With fewer vessels and narrowing of the remaining vessels, blood flow through the lungs is affected. The blood has to pass through fewer vessels, requiring higher pressure. As a result, pulmonary hypertension can develop. That is what happens in PH-ILD, although the process is quite complex.
To help visualise this, here is a sample of lung tissue taken through a biopsy. We stain the tissue and examine it under a microscope. This is a pulmonary artery. Its wall is quite thick, and the small white space in the middle is the lumen: the space through which the blood can pass. Normally, that space should be as wide as the red circle I have drawn. This illustrates the narrowing that can occur in PH-ILD.
Pulmonary hypertension is classified into five groups according to its cause and the approach to treatment. Group 3 is pulmonary hypertension associated with lung disease, which includes PH-ILD. I have just explained how that can develop. It differs from Group 1, pulmonary arterial hypertension, which primarily affects the lung blood vessels rather than the lung tissue.
Group 2 is pulmonary hypertension associated with left heart disease, which can also cause increased pressure in the lungs through a different mechanism. Group 4 includes pulmonary hypertension caused by chronic blood clots in the lungs, which block the vessels. As with ILD, pulmonary hypertension can have a range of different underlying causes.[1]
What are the symptoms of PH-ILD, and how can we recognise it? This is challenging because people with ILD or pulmonary fibrosis often already experience breathlessness and fatigue. Those are also symptoms of pulmonary hypertension. The overlap makes it difficult to recognise, both for patients and for doctors, so we need to remain alert.
Sometimes patients develop swelling of the legs and ankles, which we call oedema, as shown in the picture on the right. Some also report dizziness. However, these can be signs that the heart is already working very hard, or signs of right heart failure, which is relatively late in the disease. Earlier symptoms are often non-specific, making recognition more difficult.
Why is this important? Pulmonary hypertension is a common complication of fibrotic ILD, particularly in the later stages. Most patients who develop it have mild pulmonary hypertension. Severe pulmonary hypertension is less common, although we do see it.
Even mild pulmonary hypertension can worsen symptoms, limit daily activities and affect quality of life. Patients already have a serious underlying disease, so even a mild increase in pulmonary pressure can have a considerable impact. Early recognition is therefore important for appropriate care and treatment when pulmonary hypertension develops alongside ILD.
When should we think about PH-ILD? I have tried to illustrate this on the slide. Symptoms are difficult to interpret, as we have discussed. However, if symptoms increase without a clear explanation, particularly if they are not explained by progression of the underlying ILD or pulmonary fibrosis, we should consider pulmonary hypertension.
Relatively straightforward investigations can suggest its presence. These include an electrocardiogram, or ECG, and blood tests. In the blood, we can measure biomarkers such as NT-proBNP and BNP, which can indicate that the heart is under increased strain. Lung function tests can also provide clues, particularly the DLCO, which measures gas transfer across the lungs.
A decline in DLCO that cannot be explained by progression of the ILD may suggest pulmonary hypertension. An echocardiogram, or ultrasound of the heart, can also help. It may show enlargement of the right side of the heart or signs that it is working harder, and it can provide an estimate of the pressure in the lungs. These are relatively straightforward, non-invasive investigations.
The gold-standard investigation is right heart catheterisation. A small catheter is passed through a blood vessel, which may be accessed through the neck, arm or groin, to measure pressures in the heart. This allows us to establish whether pulmonary hypertension is present and how severe it is.
However, right heart catheterisation is not always necessary. It is an invasive investigation, so we generally perform it when the result may influence treatment decisions or outcomes. Not everyone needs to undergo it.
If someone has both pulmonary hypertension and ILD, we must also look for other possible causes of the pulmonary hypertension. As I showed in the five-group classification, left heart disease may contribute and requires a different treatment approach. Chronic blood clots in the lungs must also be excluded, as they can contribute to the raised pressure. Finding pulmonary hypertension does not mean we should stop investigating its cause.
How can we treat PH-ILD? We could devote another entire webinar to this, but my task today is to cover the basics. First, it is important to treat the underlying ILD. As I have mentioned, the treatment depends on the type of ILD or pulmonary fibrosis.
Supportive care is also important. This may include oxygen when needed, pulmonary rehabilitation and diuretics if there is fluid retention or oedema. These measures can be very helpful in relieving symptoms. Dietary advice and other supportive measures also matter. Pulmonary hypertension-specific treatments may be considered for some patients, which I will explain on the next slide.
Treatment should be tailored to each patient and provided through collaboration between ILD and PH specialists. This slide contains a complicated diagram from the pulmonary hypertension guidelines, but I will try to explain it simply. Some medicines specifically target pulmonary hypertension through the three pathways shown here. Broadly, they relax and widen the blood vessels, reducing pressure.
The aim is to make it easier for the right side of the heart to pump, so that it does not have to work as hard. That is the main effect of these medicines. In PH-ILD, there is experience and some clinical evidence for certain treatments, including sildenafil and inhaled treprostinil. Availability differs between countries, which can make the situation complicated.
We have seen that these medicines may have a positive effect on exercise capacity, particularly in patients with very severe pulmonary hypertension rather than mild pulmonary hypertension. However, not every patient responds. To understand why treatment is difficult in PH-ILD, think back to how the condition develops.[2]
A great deal of lung tissue may have been destroyed, and many blood vessels may have been lost. We can try to widen the remaining vessels with medication, but we cannot widen vessels that are no longer there. Opening narrowed vessels can also direct more blood towards damaged lung tissue.
This helps explain why people with PH-ILD may respond differently from people with Group 1 pulmonary arterial hypertension, whose lung tissue is intact. It makes pulmonary hypertension-specific treatment more difficult. Nevertheless, with some medicines we have seen improvements in exercise capacity, which is very important.
Many new drugs are being investigated, which is encouraging for the future. Several clinical trials are underway, including trials of medicines with mechanisms beyond simply widening the pulmonary vessels. We should have another webinar devoted to that.
To conclude, I want to emphasise a few points. Even within Europe, the organisation of PH and ILD care varies greatly between countries and between hospitals in the same country. Some hospitals do not have dedicated PH or ILD centres. Specialists who regularly care for patients with ILD may not have experience in pulmonary hypertension, and the reverse can also be true.
This is a challenge. Every hospital and country needs to consider how to combine and organise this care in a structured way so that patients receive the right expertise and support. I also want to emphasise the opportunities offered by clinical trials. There are important developments underway, and participation in research is important for investigating new treatment options.
My take-home messages, although you are all already at home, are that pulmonary hypertension can develop in people with ILD, especially fibrotic ILD, and that it can be difficult to recognise because symptoms overlap with those of the underlying lung disease.
Early recognition, appropriate assessment and management are important. Treatment is currently individualised, taking into account the type and severity of the ILD and the severity of the pulmonary hypertension. Multidisciplinary care involving clinicians with experience in both PH and ILD is particularly important. For everyone at home, pay attention to changes in your symptoms and discuss them with your healthcare team. Thank you for your attention. It has become quite dark here in Amsterdam in the meantime.
Louise Bouman
Thank you, Esther, for that informative presentation and for helping us understand the basics of PH-ILD. Understanding the condition is only the first step. We also need to consider how to improve awareness, ensure timely diagnosis, strengthen collaboration between healthcare professionals and provide better support for people living with PH-ILD. That is the focus of our panel discussion.
Let us start with recognising PH-ILD. You have already spoken about this, but it will remain difficult because breathlessness and fatigue overlap with the symptoms of ILD itself. What should patients and healthcare professionals look out for that might suggest pulmonary hypertension? You mentioned swelling of the ankles, but is that always a sign? Are there other signs patients can look for themselves? Esther, I will start with you.
Dr Esther Nossent
This is an important question and a major problem for both patients and doctors. When you care for patients, you remain alert to complications and changes in the disease. I always tell my patients to learn what is normal for them. If they notice a change or feel something different, they should report it. I can then investigate whether there is an explanation and whether there are signs of pulmonary hypertension.
We are particularly alert to dizziness, syncope or fainting, and oedema. However, these are already signs of right heart failure and often occur later in the disease. Symptoms like these can suggest that pulmonary hypertension has developed, but we would like to recognise it earlier.
Louise Bouman
That makes sense. Liam, what could these signs mean for patients, and how can they be recognised in practice? From a patient perspective, what might help?
Liam Galvin
Esther makes a good point: some of these symptoms appear later in the disease. At an early stage, it can be very difficult to distinguish between ILD symptoms and those of developing PH. It is important that patients become experts in their own disease. Patient organisations, primary care teams and specialists all have a role in supporting that.
Swollen ankles, dizziness and fainting are not typical symptoms of ILD, so they should immediately raise a red flag for someone living with ILD. Identifying people who are just beginning to develop mild PH is much more difficult.
There has been work on self-monitoring at home and the use of technology. In future, patients may have a better understanding of how their disease is progressing. Rather than relying solely on an instinctive feeling that they are getting worse or that something else is happening alongside their ILD, there may be ways to measure those changes. Some early work is underway, so technology may have a role in identifying PH earlier.
Louise Bouman
Thank you.
Liam Galvin
However, we are not there yet.
Louise Bouman
Patients should also feel confident that they can raise changes with their healthcare team and have their concerns taken seriously. They need to feel able to speak up rather than automatically accepting a change as simply part of living with ILD, because that is not always the explanation. That is something we need to work on as well.
Liam Galvin
We need to empower patients.
Louise Bouman
Yes.
Dr Esther Nossent
Doctors also need to remain alert. If a patient deteriorates and you cannot explain it, if the DLCO changes on lung function testing or the patient needs more oxygen, you should always ask what is happening.
Louise Bouman
Thank you. Recognising possible signs is one part of the challenge, but what should happen when a patient or their healthcare team suspects PH-ILD? Esther, when should patients seek further assessment, and what can they expect?
Dr Esther Nossent
There are several relatively simple, non-invasive tests. That is important because investigations can also be a burden for patients, and we have to find the right balance. If a doctor or patient suspects that pulmonary hypertension is developing, an ECG and blood tests are useful. Some blood markers can indicate that the heart is working harder.
A CT scan may sometimes be helpful, and an important first step is an ultrasound of the heart, which can estimate pressure in the pulmonary arteries. However, even if these tests are almost normal, they do not exclude pulmonary hypertension. That makes diagnosis difficult. We need to consider all the investigations together with the clinical signs.
Right heart catheterisation is invasive, although it is a very safe procedure. We do not see many complications in our centre. Nevertheless, we should only perform it if the result will have consequences for treatment decisions or outcomes. In that situation, right heart catheterisation can establish the diagnosis.
Louise Bouman
That is very clear. Liam, could you add the patient perspective and explain the importance of clear communication?
Liam Galvin
When patients are diagnosed with ILD, they should be made more aware of the possibility of PH developing alongside it and the symptoms to look out for. People with ILD already attend hospital regularly. They might have a six-minute walk test or lung function tests, but an ECG or heart ultrasound is not necessarily part of those appointments.
Perhaps we need to work with healthcare professionals to embed awareness of PH into ILD clinics. Guidelines have a role, as does making sure that non-specialist centres caring for people with ILD are aware of the possibility of PH. Clear communication matters because, if patients hear about this when they are diagnosed, they know which symptoms to look for. Hopefully, that will help identify it earlier.
Dr Esther Nossent
May I add something, Louise?
Louise Bouman
Yes, of course.
Dr Esther Nossent
I really like that point, Liam. It makes me consider whether I always explain to my patients with ILD that they may develop pulmonary hypertension. I do not think everyone does, partly because we cannot know whether an individual patient will develop it. Nevertheless, raising awareness is important.
There is also a distinction between early recognition and routine screening. Screening means building regular investigations into care pathways to identify pulmonary hypertension early, but those investigations also place a burden on the patient. If we introduce routine screening, using the algorithms now being developed, it should also improve patient outcomes. That is important. Work is underway, and some algorithms are being developed.
Louise Bouman
That would be very helpful. Earlier recognition is better when there is something available to treat the PH-ILD, and hopefully we will not have to wait too long for that. Clear communication is essential: patients need to understand why further tests are being considered, what they involve and what the next steps will be.
Early recognition matters, but so does ensuring that patients are not left in uncertainty while waiting for answers. That can be a horrible position to be in. Waiting for results can feel endless, and you start thinking, “Is it getting worse? Am I getting worse? Am I going to die?” All kinds of frightening scenarios can go through your mind. Knowing what to expect and how long you may have to wait can make a difference.
That is something we should address. Thank you both. Getting the right diagnosis is important, but it is only one part of the journey. Once PH-ILD is suspected or diagnosed, collaboration between healthcare professionals becomes crucial to ensuring that patients receive the right care at the right time.
How can pulmonologists, whether they specialise in ILD or PH, and other healthcare professionals work together more effectively? Esther, perhaps you could start with collaboration between ILD and PH teams.
Dr Esther Nossent
It is a challenge, and arrangements differ between countries and hospitals, including hospitals within the same country. We have to ensure that patients receive the right care and expertise. Professionals do not have to work in the same hospital to collaborate well.
Hospitals can organise multidisciplinary team discussions, or MDTs, including by video. There are various ways to arrange this. Collaboration with patient organisations within each country is also important, so that everyone recognises the need to organise care well. It involves not only doctors but also specialised nurses. Their involvement is important too.
Louise Bouman
Liam, could you explain the role of patient organisations further and how coordination can be improved from the patient’s perspective?
Liam Galvin
That is an important point. Like hospitals and specialist centres, patient organisations can be somewhat disconnected. We are fortunate that the pulmonary hypertension and pulmonary fibrosis communities have worked closely together for many years because of the overlap between the conditions. I do not think that has necessarily been the case for specialist consultants.
I was surprised and pleased to see that Esther works in a centre specialising in pulmonary hypertension, ILD and sarcoidosis. As she said, arrangements vary between countries, and many do not bring these services together in that way.
She also mentioned MDTs. I think these are increasingly recognised as the gold standard for diagnosis. In many countries, an MDT might include a rheumatologist, pulmonologist and cardiologist reviewing a particular case. For example, a rheumatologist may contribute to diagnosing ILD associated with rheumatoid arthritis.
Where we are lacking, and where patient organisations need to help change healthcare systems, is ensuring that MDTs are involved not only in diagnosis but also in ongoing clinical care throughout the patient journey. Patient organisations need to work closely together. Although our diseases differ, there is overlap, just as there is overlap in the symptoms.
There is overlap in the work we need to do, including encouraging good practice such as bringing specialists in different diseases together under one roof. Patients may otherwise need to see a cardiologist for aspects of their PH and then travel to a different hospital for their ILD care. That does not make much sense and places a burden on patients. This is where organisations like ours may need to step up our efforts.
Louise Bouman
I agree. We need better communication between specialists, clear referral pathways and clarity about who coordinates care. Multidisciplinary teams already exist, but patients do not always know who leads their care or who will communicate decisions to them. That can be difficult, and those responsibilities should be clear.
Patient organisations can also help people understand their condition, prepare for appointments and decide what to ask, and find reliable information and peer support. These forms of support can be particularly helpful. Good collaboration should mean that patients experience one coordinated care journey rather than a series of disconnected appointments.
We have talked about the medical aspects of PH-ILD, but living with the condition involves much more than medical treatment. Liam, could you start by sharing what you see as the biggest unmet needs for patients and their families?
Liam Galvin
Looking at PH and ILD as a whole, with all their variations, I think many of the problems and unmet needs are similar. We can usually access medical treatment and see specialists, although the quality of that journey varies. What is often missing in many countries is practical and psychological support, and access to some of the supportive measures Esther mentioned.
For example, patients are not referred to pulmonary rehabilitation in every country, and there may be issues with the cost of oxygen. For me, anxiety and the psychological burden are major issues. Social care or support from a social worker can also be important, because the disease has financial consequences. A carer may need to take time off work.
The broader impact of the disease, including its effect on individual families, is often overlooked. The fact that it is rare adds to that burden. Patients repeatedly have to explain their condition. Primary care doctors and the public may know little about pulmonary hypertension or pulmonary fibrosis, and even less about the combination of the two.
Louise Bouman
The burden is greater than people often expect. Patients need more than medical treatment. They need appropriate rehabilitation, which is not always available, supportive care, psychological support when needed, clear information and opportunities to connect with others who understand what they are going through. Esther, what can healthcare professionals do to help with the burden beyond medical treatment?
Dr Esther Nossent
Do you mean the non-medical burden?
Louise Bouman
Some of it is medical, but some is not. It is about living with the condition every day and wanting to get a little of your life back. That is difficult and takes hard work. What can healthcare professionals do to help with that?
Dr Esther Nossent
As Liam said, it is important for doctors to recognise these issues and pay attention to them within clinical care pathways. That includes psychological support. I began the presentation by saying that we can improve test results or measurements, but that is not all that matters. It is about quality of life: how someone wants to live, what matters to them and what could improve their life. The answer differs for every patient.
We also need to consider the impact on families and carers. For example, in our hospital we sometimes organise information evenings for carers, giving them information and an opportunity to discuss their experiences. We also offer psychological support and training to help them.
Needs differ between patients, but we can build attention to these issues into clinical care pathways. Healthcare professionals do have a role in addressing them.
Louise Bouman
Thank you. We need to treat the person living with PH-ILD, not just the disease, and ask patients what matters most to them rather than assuming we already know. That assumption can be a pitfall.
I would like to end our discussion by looking ahead. We have talked about recognition, diagnosis, collaboration and the challenges of living with PH-ILD. If each of you could change one thing today to improve the patient journey, what would it be? Let us begin with the patient perspective. Liam, what are your thoughts?
Liam Galvin
For me, it is awareness. As I mentioned, living with a rare disease is difficult, particularly with an even rarer condition such as PH-ILD. There is work to do in raising awareness so that people and healthcare systems understand the burden and can adapt to provide better support.
Outside the medical aspects, awareness is what I would most like to change. I want people to understand the burden, know how they could help, or simply be more considerate of patients and their families. Awareness would be my choice, Louise.
Louise Bouman
That is a good one. What about you, Esther?
Dr Esther Nossent
Awareness is very important. From a medical perspective, I also think we need to understand what is clinically relevant when developing new medicines and trials. What should our clinical trial outcomes be? How can we improve patients’ lives, rather than focusing only on the disease?
We should integrate that into trial design, drug development and treatment. I think this is a major unmet need at the moment, and it is what I would like to change.
Louise Bouman
Thank you. My answer fits with both of yours. I would like to establish a clear pathway for people with ILD in whom PH is suspected. Patients should know when to seek help, healthcare professionals should know when to investigate further, and people should be able to access the right expertise without unnecessary delays. That remains a major hurdle.
Ultimately, we need to move from simply listening to patients to involving them in shaping their own care. Patients understand the daily burden of their disease. The patient voice should not just inform change; it should help drive it.
Thank you, Esther and Liam, for this valuable discussion and for sharing the clinical and patient perspectives. There is much more to discuss, so we would now like to turn to our audience and address as many questions as we can. I will check the chat. I can see one question: “What percentage of people with ILD have PH?” Esther, that is a question for you.
Dr Esther Nossent
It is an important but difficult question. There are many registries and trials, but PH is not always defined or measured in the same way. Some studies use right heart catheterisation, while others use echocardiography. There are also different types of ILD, which makes comparisons difficult.
In the later stages of severe fibrotic ILD, a high proportion of patients, perhaps around 70%, may develop mild pulmonary hypertension. We estimate that fewer than 5% develop very severe pulmonary hypertension. However, I need to be careful with these figures because registries and trials use different definitions, and cases are not always confirmed by right heart catheterisation. A large European registry study will hopefully give us a clearer answer in the next few years.
Louise Bouman
Thank you. It has not always been actively looked for, partly because there were no treatment options. That makes it difficult to get a clear picture.
Dr Esther Nossent
Exactly. In the past, PH in patients with ILD was not always confirmed by right heart catheterisation because there were no treatment options. The investigation was often not performed, which makes the numbers difficult to interpret.
Louise Bouman
I do not see any other questions at the moment, although there are messages thanking Esther for an informative presentation. If you have further questions, please put them in the chat. It is exactly seven o’clock, so we are on time, but we can still discuss any questions that come in. Otherwise, I would like to thank everyone for joining us.
There is another comment: “Thank you for a very interesting webinar and for stressing the carer’s important role in the patient journey.” That is very true. Carers are often forgotten, but they may notice changes in how someone is living even before the person notices them. Patients may keep going and overlook changes, while carers see what is happening and worry, sometimes more than the patients themselves.
Carers therefore have an important role. They can be very honest with patients about what they observe, and that honesty matters. Thank you for highlighting that.
Thank you, everyone, for attending, and thank you for the presentation and discussion. I enjoyed the discussion, and I hope people have learned from it. I hope we can provide more webinars like this together. Thank you, and have a good evening.
Liam Galvin
Thanks, everyone.
Dr Esther Nossent
Thank you.