Sarcoidosis and the Lungs – 2025 October Sarcoidosis Patient Day

Transcript 

Graham Bloye: We’re going to move on to our first speaker now. We know that pulmonary sarcoidosis is one of the most common forms of the disease and it will be affecting many of you who are with us today. So, we’ve invited Professor Robina Coker to talk about sarcoidosis and the lung this morning. Now, Rubina has been a very good friend of the charity for many years. She’s also a member of our Medical and Scientific Advisory Panel. And she was also involved in the research decision that Dawn has spoken about just now.

Robina’s day job is working as a respiratory consultant at Hammersmith and Charing Cross Hospitals. That’s now part of the Imperial College Healthcare NHS Trust. She is highly qualified and takes a very strong interest in interstitial lung disease, including sarcoidosis, and she had a major role in establishing the British Thoracic Society’s Interstitial Lung Disease Registry that now holds over 3,000 patient records across 60 centres across the UK. It’s a very, very important registry.

So, thank you so much for giving up your time today, it’s really appreciated.

Professor Robina Coker: Thank you very much, Graham. Good morning, everyone, and thank you very much to Graham, Sue, and Dawn, and everyone else on the committee for inviting me to speak to you today. So thank you very much for that introduction Graham, and I’ve been asked to talk about sarcoidosis and the lungs, and I will pretty much stick to that.

We’ve got an hour and a half, which is quite a long time, and I’m slightly concerned you’re going to get very tired of listening to my voice, but I realise the format makes interaction quite tricky. I’m hoping to go quite slowly through these slides, and to try and demystify some things for people. Now, I realise that people joining may be very much at the start of their journey with sarcoidosis, or they may be quite experienced –  they may be very experienced.

So everybody may have different levels of knowledge, so some of the things I say, you may think, yeah, yeah, well, I know that, we should move on, but bear with us, because I think it’s right that we bring everybody up to speed, and we just help people to feel reassured about what happens with the diagnosis of sarcoidosis, how we make it, and how we then go through possible management. There will be things, as I say, that some people will be very familiar with.

The other thing I’d say is that we’ve had some fantastic questions submitted in advance, which I’m very grateful for because it’s really useful to know what sort of things people are thinking about.

Now, some of those questions, I think I’m going to cover in my slides, in my talk.

Some of them are not directly related to respiratory, but I’m happy to try and just cover them a little bit from my perspective, bearing in mind that I am only a lung disease specialist, and I’m not an expert in everything else. Although sarcoidosis of course is and can affect anywhere. So I’ll talk about some of the general questions, if I may, afterwards.

Then we’ll take it from there. And I think, Graham, you’re proposing also to have a sort of more dedicated open question session at the end, is that right, towards the end?

Graham: That is correct. Simon Hart and Nik [Hirani] will be answering an hour’s worth of anything to do with sarcoidosis –  anything goes, basically.

Robina: That’s later on that’s after the break. Okay, fine. So I thought I would just begin, and this is nothing to do, really, with the lungs so much, but just to refresh people’s memory of sarcoid, and what it is, and when it was discovered, and when we think it was discovered. This esteemed gentleman, this portrait on the left is Jonathan Hutchinson, he’s British, he was British. He was credited with the first reports of sarcoidosis in this country, although, to be fair, there were other people in Scandinavia and the USA working on this, and probably there are a number of simultaneous reports.

You’re thinking about the late 1800s, early 1900s really, for sort of first case reports and descriptions. And actually, interestingly, it was often,  dermatologists, so skin doctors who got to see sarcoidosis first, because people presented with skin rashes, so that’s quite interesting, probably because, there was very much less known about the lungs, and people didn’t have access in the same way to chest x-rays, and certainly not CT scanning. That’s what happened. And what about who gets sarcoid, and how common is it? I mean, typically, it’s taught, and your average medical student will tell you this, but it’s adults aged 20 to 40. Now, it’s become very clear through the sarcoid registry, which Graham talked about earlier and through a number of other studies, including a big case control study in North America about 20 years ago called the ACCESS study that actually, although, yes, it does affect younger adults, it can also present in older adults, and you cannot rule out sarcoidosis just because somebody is older when they first developed symptoms. So that’s really important. Generally, we don’t see it in children, on the whole, okay?

Now, it’s quite difficult to ascertain how common sarcoid is because very often people don’t have symptoms, so we don’t pick them up. So we don’t know how many people are out there in the general population who might have sarcoidosis, and that’s really tricky. But we think in the UK, there are about 10 to 20 people per 100,000.

It’s more common in certain groups. So it’s more common in Scandinavian countries. It’s more common in Irish populations, and it’s more common in Black and Afro-Caribbean populations.

And there are some genetic links, there’s some links, there’s lots of studies with HLA genes showing some predispositions. These are genes which are involved in the immune response, so the response our bodies make to foreign proteins. But they’re not clear-cut, there’s no easy solution to this. They’re not direct genetic links, and there’s no one gene that determines sarcoidosis, so it’s a very mixed picture, and the studies are really quite tricky to unpick, and they don’t help us very much in predicting who’s going to get sarcoid, who’s going to get it badly, and so on.

So they’re interesting, but that’s just to note, really. What is important, and this depends really where doctors and nurses and teams looking after patients with sarcoid are working, and where you might live, is that Black and Afro-Caribbean patients often have more severe disease. And they’re at greater risk of extra-pulmonary manifestations. So they’re at greater risk of disease that doesn’t affect the lungs. So where you have clinics with a lot of those groups in there, and that includes the, as I say, the Irish and the Scandinavian populations then you may well be dealing with a much more severely affected population. So that’s also really important.

That advanced a little earlier than I thought it was going to. So, I’ve talked generally  about sarcoidosis, and now I want to focus on the lungs, which is what I’ve been asked to talk about,  and so why are the lungs so important? What is it that makes the lungs important in sarcoidosis?

Well, the first thing to say is that over 90% of people that we see with sarcoidosis will have lung involvement of some kind and sarcoidosis is the second commonest long-term lung condition in adults. So even though it’s not a very common condition, and most general practitioners may only see one or two cases during the course of their career, it is the second commonest long-term lung condition. The first commonest, the top one, of course, is asthma. So it is important from that perspective. And it means that many, if not most people with sarcoidosis will see a respiratory, a lung specialist, at some point during their journey.

There are caveats to that, so people may have limited skin or eye involvement. They may not get to see a lung specialist, but very often, lung specialists have a bit of a reputation for doing this sort of coordination job with their colleagues and referring and signposting people to the specialists they need to see. So, very often, lung specialists get the experience in sarcoidosis, and they end up running sarcoidosis clinics. That’s how that arises. Now, it’s really important to know and to recognise that not all lung involvement by sarcoidosis is the same. There’s lots of different manifestations. And I wanted to unpick something that you may, as a patient or a carer or a friend, have seen or family member, in a letter, and thought, I wonder what on earth that means. And doctors sometimes describe different stages of sarcoidosis in the lung. And they won’t necessarily mention Scadding, but they might, because if they talk about stages, they’re actually referring to this chap, okay, Scadding. And I’m just going to spend a couple of minutes on the history, because it’s quite fun. And it just gives you a little bit of background to what John Guyett Scadding was doing.

So, he was actually born in the early 1900s and died in 1999. He was a very highly qualified doctor. He did, as was fairly common in those days, both medical and surgical posts, and he worked at the Brompton Hospital in Chelsea for a number of years, and he was rather appalled that hospital doctors didn’t get any teaching at that stage and so he was quite passionate about this. And he was later, in 1935, appointed as a consultant at the British Postgraduate Medical School, being developed at Hammersmith Hospital, and that’s where I work.

When I joined the Hammersmith Hospital a long time ago, it was still called part of the Royal Postgraduate Medical School, which has since been absorbed into Imperial College London. And then four years later,  he got a permanent post at the Brompton, and then he continued at the Hammersmith part-time, so he sort of worked between the two hospitals, and there have been quite close links between the Hammersmith and the Brompton over the years, with I’d say some sort of friendly competition and discussion, and quite a lot of collaboration, particularly in research. So, it’s really good that we can sort of continue that tradition.

He did all sorts of things. He was very busy, and he had a very illustrious career, and he’s really given the credit for launching respiratory medicine as an academic specialty in the United Kingdom, and this is really relevant, because Dawn’s been talking about the fact that if we don’t have really good quality research, and we don’t have academics and researchers working in lung disease, we’re not going to get the answers, we’re not going to get the new treatments. So it’s really great to have good management and good communicators and doctors and nurses who can manage the day-to-day problems of patients. But unless we have research, and unless we have questions being answered, we really can’t develop and improve. So, you know, this was really key, and it continues to be really important. He founded an academic lung society called the Thoracic Society. They then produced a journal, Thorax, which is now one of the leading international journals in respiratory medicine.

He was appointed president of the British Tuberculosis Association, and he later brought that society together with the Thoracic Society and founded the British Thoracic Society, and that is now the professional organszation for lung specialists and healthcare professionals in the UK. So he really had a very illustrious career.

Now, as part of all that, he was quite busy with sarcoidosis. He didn’t think sarcoidosis was properly described or understood, and you may still think that’s the case, and there’s undoubtedly still a lot of things we don’t know, which enhance the importance of the research.

But one of the key things he did was, and of course, remember, he didn’t really have access to CT scans very much, was he described the chest x-ray findings and he categorised them. And there are people who think this categorisation, because it dates back to the 1950s and 60s, is obsolete now because we have access to high-resolution CT, and I’ll come on to high-resolution CT later.

But nevertheless, it’s a really useful, quick way to describe sarcoidosis. We use it in the sarcoidosis registry because it’s quick and easy and it gives you some idea of what’s going on with the patient and the lungs. And these percentages here describe the percentage of people who will have this pictured, on average, when they present with sarcoidosis.

So, a relatively small number of people have a normal chest x-ray when they present, and that’s probably because when people have an abnormal chest x-ray, they get referred, but you know, if you have a normal chest x-ray, nobody necessarily thinks there’s anything wrong with you. So it’s probably skewed, but anyway, about 5% have a normal chest x-ray. And you can see that half of patients have this funny thing called BHL, which is bilateral hilar lymphadenopathy, which is called Stage 1, and that is where the glands in the centre of the chest X-ray around the centre of the lungs are enlarged, and I’ll show you a picture of that in a minute, so you know what I’m talking about. And then, Stage 2, is where you also get some inflammation or infiltrate sort of fluffy shadowing in the lungs. And then you may have, people with Stage 3 who’ve just got the fluffy shadowing, and they’ve lost the glands.  And then a small percentage of people go on to develop fibrosis, which is scarring of the lungs, and the thing about scarring is it’s irreversible.

Now, it tends in sarcoidosis to affect the upper lobes of the lungs, which is good news, because the upper lobes actually don’t do as much work as the lower lobes. They’re much smaller, and you can manage without your upper lobes. You might not be an Olympic athlete, but you will be able to manage without your upper lobes. So actually, if just the upper nodes are affected, you should still be okay. But the fibrosis, once you’ve got it, is irreversible.

Now, people do not necessarily progress through these stages. You can regress from stage 1, 2 or 3. You improve, and you can go back to normal. Those 3, or 4, if you like, do not indicate necessarily a progression, and it’s not a sort of cancer staging, because I think people sometimes get a bit confused, and they see the staging on their letters, and they think, well, oh dear, what’s going on? What’s happening?

This is a little bit blurred, I think, on your screens, but this is a chest x-ray of a lady with these enlarged glands on the chest x-ray. You can see they’re quite lobulated and rounded, and if you have a pattern like that, ask your doctor just to show you your chest x-ray, because they’ve probably got digital access in their clinic room and they can just show you the picture quite quickly, and it just gives you an idea of what people are talking about, because I do think when you get things on letters you don’t know what they mean and they can be quite concerning, and there’s an awful lot of jargon in the letters. Inevitably, because that’s how doctors code things and communicate with each other, but it can be a bit tricky, so do ask. But the lungs actually here look nice and normal and nice and black. There’s no white shadows, really, there’s just a little bit of structure there. They look very normal, so that’s what we like to see, really. And then this one is a bit different. This left lung is much smaller than the right. There’s quite a lot of white, sort of linear scarring in the upper lobes, and there’s this little rounded shadow here, which is actually a fungus ball. So this is somebody with S tage 4 sarcoidosis, so fibrosis and scarring of the lungs. They are very different. Okay, so that’s a little bit about staging and where that came from.

Now, you’re probably all familiar with the symptoms of sarcoid in the lungs, so cough and breathlessness. Chest pain’s a tricky one. I think there were one or two questions about chest pain. I’ll come back to that, and if I don’t, remind me, because chest pain, we don’t always understand, and it’s tricky, and it can also be a sign of cardiac sarcoidosis. I’m not sure about just including chest pain, but I will just for the moment.

The question that comes up very often is, well, how do we measure lung involvement in sarcoidosis? Because it’s really tricky. And one of the things that you may have found frustrating during your journey is the time it took to get the diagnosis of sarcoidosis. An improved diagnostics is definitely something, I think, for the research questions, and it was the theme of some of the research proposals that we looked at a couple of weeks ago.

Because, as I explain to patients, but also my students,  there is no single diagnostic test for sarcoidosis, so I cannot just take a breath test from you or, do a blood test, and say, yep, it’s sarcoid, and there’s nothing else it could possibly be.

And so it is really tricky. And measuring lung involvement is also tricky. So, there’s all sorts of things that we do to try to work out how badly the lungs are involved, or if they’re involved at all. So, symptoms are really important.

So breathlessness is important, but, and I’ll come on to this when I talk about some of the questions, breathlessness, of course, is a very complex symptom. We don’t actually understand all the causes, all the physiology of breathlessness, and there’s lots of people researching that separately from sarcoid.

And there are all sorts of other things that can make people breathless. You might have a heart problem, or you may not. You might have put on a bit of weight recently.

You might be quite anxious or stressed, and you might have got a bit of a breathing pattern disorder. You might have weak muscles, and we’ll come on to that, so you might be what we call “deconditioned”. So, in common parlance, that probably means that you’re a bit unfit.

So, there’s all sorts of things that contribute to breathlessness. So, breathlessness is important, but it’s not always a good, reliable measure of involvement in the lungs by sarcoidosis. We try and narrow this down with questionnaires sometimes to try and get objective pictures so that we can measure, sort of, serial progress. There’s a fairly rough and ready, but again, quite useful Medical Research Council Dyspnoea Scale, which goes from 1 to 5. 1 is no problems, and 5 is housebound. So, you know, you can see it’s fairly crude, but it’s quite a good description. Then we do things like breathing tests, or lung function –  I’ll come on to those in a minute, and then we do imaging. And chest X-ray is very useful –  very low radiation exposure, so it’s very safe. We can do quite a few x-rays in a year and not worry too much.

High-resolution CT has a little bit more radiation exposure, but it’s going down all the time because the scanners are getting more and more sophisticated. And high-resolution CT scan is what we’re talking about here with really fine sections. So 1mm sections at intervals and 10mm intervals through the lung fields. So we’re sampling the lungs, so it assumes that the process we’re looking at is a diffuse process with scattered changes throughout the lungs. And it’s it’s not going to pick up everything in the lungs, but it will pick up sections, and it will give you crystal clear pictures, so it’s really, really nice for looking at the lungs in great detail in that situation, it gives us a fabulous amount of information, and sometimes it gives us information we don’t know what to do with, because we pick things up and we don’t know the significance. And I can come on to that later as well.

So what happens during lung function? This is where some of you, the experts, you probably can switch off for a moment, but do please stay tuned so you come back. So you’ll get these sorts of instructions beforehand, loose clothing, well, yes, because you don’t really want to be wearing your tightest clothing when you’re exercising. You don’t want to be doing lots of forced blowing tests when you’ve just had a large meal or exercised very heavily, because you’ll be absolutely exhausted by the time you get to the lung function lab. You don’t want to be smoking, you shouldn’t be smoking anyway. I’ll talk about that later, and we don’t recommend that you drink alcohol beforehand. The lung function teams are quite good at picking all these things up. So just be aware that they’re very expert physiologists.

So they will pick up if people haven’t followed the advice.

You have to do these things with a nose clip, which some people find quite uncomfortable and stressful, and it definitely gets easier with practice, so I do have patients who don’t do very good lung function initially, and everybody gets a bit concerned, including the patient, because they think, well, the results aren’t very good and we say, well, let’s try it again in a few months, and actually it’s much better, because you’ve had a chance to practice. So generally, it may improve a bit with practice if you find it difficult first time.

You do these tests sitting down, generally, you’re given a disposable mouthpiece to use, and you have to put that in your mouth. Again, that can be a bit uncomfortable, and you have to sort of form a tight seal over it with your lips. And then you’re asked to do various breathing manoeuvres while you’re connected to a device that’s recording all sorts of things, and that can include deep breathing in and out, holding your breath. Breathing as hard and fast as you can. You might have to pant in a closed cabinet, and then you might, in some cases, you might be asked to use an inhaler, usually the blue Ventolin inhaler that some of you will be very familiar with, to see whether that changes your results.

And so, specific tests include things like spirometry, blowing tests where you have to take a deep breath and then blow out as hard and fast as you can. You’re told until your lungs are empty, actually, your lungs are never empty, but that’s how it feels.

Or you might, as I say, have to sit in a closed booth and breathe normally and then pant. You might have to be able to breathe…asked to breathe in and out normally and hold your breath for a short time, or breathe out against a suctioning action. And these tests really can quite exhausting. Some people sail through them, some people find them really difficult, and find them very exhausting, and some people really don’t like doing them.

And there are other things we can do if people really don’t like doing these tests, we can get some idea of your functional capacity by getting you to do what’s called a 6-minute walk test. We walk you up and down a corridor, and we see what happens to your oxygen levels, and how far you can walk during that time. So that can be quite useful. But if you’re fairly fit you’ll probably find it too easy, so it’s only useful when you’re not quite so well.

And then the other thing we can do, we can do slow breathing, and people often find that a bit easier. So, there are ways we can adapt this, but generally, we like to have these forced manoeuvres if we can, because it gives us a really good picture of what’s going on with the physiology and the lung function. Now, I was asked a question, a very good question, about diffusion, oxygen diffusion, when it becomes a concern, and what you can do to improve this. So I put this slide in just to remind me to address that.

Now, one of the things we measure during lung function is gas exchange or diffusion. That’s a bit of a technical term, isn’t it? What it’s talking about, really, is the efficiency of the lungs in taking up oxygen from the air that you breathe into the circulation.  This section here on the left shows you a section, a microscopic section, cross-section, of normal lung tissue. So where that little black arrow is sitting is what’s called an alveolus, or an air sac. And the oxygen comes in in the air that you breathe down through your airways into the alveolus.

And it’s a passive process, it just diffuses down a concentration gradient into these red cells here. You can see them really nicely, which are sitting in these little capillaries, little blood vessels.

And they’re waiting, they’re short of oxygen, they’ve come back from the body, they’ve come back to the lungs for oxygen – they want to pick up oxygen. So they’ve got very low concentration of oxygen. And the alveolus, the air sac, has got a very high concentration of oxygen. There’s no pump doing this, it’s just diffusion. So, I sometimes liken it to the process if you have an old-fashioned sink with old-fashioned taps, and you have a hot tap on the left and a cold tap on the right. If you pour vigorously from both taps with the plug in, and you fill the sink, and you don’t agitate the water, you just leave it dip your hand in either side. The left side will tend to be hot, and the right side will be cold. But if you come back a little while later, you will find that actually the water is tepid. And that’s because down at the diffusion gradient, the hot molecules have gone to the right, and so on. There’s been an exchange down he diffusion gradient which is exactly what happens here. It’s the same process. This process, to be effective, relies on these very fine alveolar membranes. You can see they’re one to two microns thick, micrometers thick. So hopefully that’s explained to you what is happening all the time when you breathe, 12 to 16 breaths a minute, okay?

Right. Now, this is a cross-section of scarred lung. Now, this actually does not come from a patient with sarcoidosis, but this is a patient with very severe scarring. And you can see that all those lovely air sacs, these alveoli, have just been replaced with this horrible, thick scarred fibrous tissue. It’s stained with a particular stain. But basically, this is all just collagen. Scar tissue. So the same sort of scar tissue that you get if you cut yourself badly and it doesn’t heal invisibly, alright? So, basically, the oxygen has great difficulty, then, in getting in, because there is no pump. And it’s a big molecule of oxygen, and it just struggles, because these membranes have been obliterated with scar tissue.

Now, usually, as I say, you’ve got upper lobe involvement, with sarcoidosis, and the lower lobes are still working well.

So it isn’t too much of a problem. Usually it’s not a problem at rest, you’re okay when you’re sitting, but if you, and it’s a very small proportion of patients with sarcoidosis, who will get this badly, but if you get really bad scarring, then it can be a problem when you exercise, and you can drop your extra oxygen levels. So going back to our lovely question about what percentage oxygen diffusion becomes a concern, and what action can be taken to improve this? There’s a couple of things I’d say.

Diffusion, generally, anything is like 60%, we’re sort of okay with. We’re not really worried about. It’s when it gets down to about 40% we get a bit more concerned. By that stage, people are usually quite breathless. And at that stage, we would be thinking about more advanced treatments, and I’m coming on to treatment later, so we’ll come back to that. But 40% for diffusion is sort of one of those figures we have in mind, that we’re 45 or 40%, we’re getting a little bit concerned. Now, I wasn’t sure whether the questioner also had in mind things like oxygen saturations, which are slightly different. This is measuring the percentage of oxygen in the blood. And usually, again, at rest, we expect that to be about, sort of, above  98% or above.

When that becomes a problem, for instance, on exercise. So I talked about walking people up and down a corridor, doing a six-minute walk test. If that falls below 90%. then we think, actually, are there other treatments we should be thinking about? And I’m going to come on to that later, but I hope that answers that question. There was a supplementary question to that, which was very sensible, which is, what action can be taken to improve this?

Well, one of the things we can do, apart from treating the sarcoidosis and trying to prevent further fibrosis, because you remember I said that fibrosis isn’t technically reversible, is what you can do is you can just make sure you’re not anaemic. Because one of the limiting factors for this process, as you can see very nicely from here is the number of red cells.

All right, so the red cells are like taxis. They come in, they pick up the oxygen, they go back to the heart, and they go around the body again for their next cycle. So the oxygen molecules are like passengers in a taxi. If you haven’t got enough red cells, if you’re anaemic, that will affect the process. So sometimes, if you’ve got very mild anaemia, it’s not going to be terribly important, but sometimes it’s worth thinking about, do we need to correct anaemia? So that’s always just worth bearing in mind.

Okay, now I’m going to carry on. So we talked quite a lot about lung function. High resolution CT, some of you here will be experts on this. There’s other people sort of say, oh, what’s the high-resolution CT again? What do I have to do? Is it claustrophobic? Well, not really, because you lie flat on admittedly, not a very comfortable couch, but you will have some kind of pillow, that moves through a donut-shaped machine, and it takes lots of different pictures from lots of different angles, 360 degrees. It lasts up to 30 minutes. 30 minutes is usually quite long. They’re usually quicker than that. You might be asked to hold your breath sometimes, just to get really clear pictures. You’ll have a technician who’s communicating with you from another room, because they don’t want to get irradiated, because they’re doing it all day, so they don’t want to be irradiated all the time. It’s not that you are getting a particularly big dose. And then after that scan, a radiologist or specialist in x-rays and CTs will look at the images and “write” in inverted commas, because nobody writes anything now they dictate, a report. So that’s what happens with high resolution CT.

So, how do we prove or diagnose lung involvement? So we would look at the x-rays, and I’ve shown you some examples before. We would probably then go on to CT, and the typical features are captured here for you. You’ve still got these enlarged glands in the centre, which I talked about, so these white, sort of rounded handles sticking out, but then look at all these little modules here, and they’re scattered along the fissures. So this is called “beading”, and it just means that these are supposed to look like beads along the string of a necklace, and those are actually granulomas, so those are inflammation. I’m talking about granulomas in a minute –  what are they?

But these are the size of them as seen on the CT, but lots and lots of nodules affecting the lungs. So that’s a typical CT. And then, you’ll probably have a discussion when you’re diagnosed with your… or when you’re approaching diagnosis, with your specialist about how to get a tissue diagnosis, and this is because, if you remember, I said there’s no specific diagnostic test for sarcoidosis. So what we had to do is we had to have to get as many clues as possible, put them all together, a bit like a detective. And then we have to rule out other causes, because there are some conditions that famously mimic sarcoidosis, and you really don’t want to mix them up. So we do have to be quite careful about ruling out other diagnoses, and it may seem very tedious and very long-winded for you when you’re going through this process, and you may find it exasperating, but it is actually really important to do this properly.

So sometimes we do a bronchoscopy, so we do a telescopic examination of the lungs with some sedation. So it’s a very fine tube with a flexible tube, the bright light on the end of it, and it goes down into the lungs. And you’ll have some sort of valium-type medicine, just to make you a bit sleepy, so it’s not too uncomfortable, or so that it doesn’t make you cough. We can have a good look, and we can sometimes take some biopsies. A more sensitive test is the EBUS test, which is like a bronchoscopy. It also involves ultrasound screening and a needle put into some of the glands that are enlarged that we can see on CT. It takes a bit longer, you need a bit more sedation, it needs somebody who’s experienced doing it, so it’s usually only practiced in certain centres.

But it does have a higher yield, and that became very clear from the sarcoidosis registry, and it is probably now the commonest way of diagnosing sarcoid, because this pattern of enlarged glands is so common.

Sometimes you don’t have the enlarged glands, or you really can’t get the diagnosis, and you have to go to surgical lung biopsy. That’s tiresome, but having said that, thoracic surgeons are getting very clever now at doing minimally invasive lung surgeries, so it isn’t such a big deal as it used to be and people can be out of hospital in 2 or 3 days. But it’s not trivial surgery, so we try to avoid that wherever possible. We try to do the least invasive procedure. And sometimes it will be possible to make a diagnosis from a gland in the neck or in the armpit, or in the groin. Or it might be that you’ve had a liver biopsy. So we don’t always have to biopsy the lungs, but the reason for putting this up, of course, was that I was asked specifically about the lungs, so that’s what I’ve tried to do.

Now, when we talk about granulomas, what on earth are we talking about? Well, we’re talking about this pattern here on the microscope. It’s a collection of inflammatory cells. You’ll just have to trust me that there’s all sorts of cells in here called T cells, which help regulate the immune response. And macrophages, which are inflammatory cells, and they cluster around in a very characteristic sort of elliptical fashion, and it’s a very, very classic picture. However, TB can also do this and typically, TB causes necrosis in the centre. So, death of cells and destruction in the centre, and it will look a bit different. This one doesn’t have any death or destruction of the cells. So this is called non-necrotic.

Now, people may see on their letters or notes, they may see the term caseating, and they may think, goodness, what on earth is that? And as I explain to my students and my patients quite a lot, a lot of the terms we have in medicine we still use, are archaic, frankly, which is why we’ve moved more to using necrotic. When 19th century pathologists were looking at all these things they often used terms like, words related to food and things that they were familiar in general terms, and so “caseating” came from caseous, or cheese, and it came from the appearance of the centre of ripe cheese, sort of liquifying and falling apart, which isn’t really very pleasant. So I think we’ll stick with necrotic on the whole, but you may see this term, so it’s worth just knowing what it is. It isn’t something really, really horrible, it just means there’s some death and destruction in the centre. Now, classically, sarcoid doesn’t cause that. But there is no never in medicine, so you can be confused.

You can get coexistent TB. So, you really, really have to rule out TB, because the first treatment, first-line treatment for sarcoid, as you all know probably, is steroids, which suppress the immune response, and the one thing on the whole you don’t want to do in TB is to suppress the immune response, unless you are already treating the TB actively with anti-TB medication. So you can add on steroids, that’s sometimes done, but you don’t want to treat TB just with steroids, because you’ll make the patient very ill. So, we have to try and rule out TB as far as possible, so we do special stains to look for the TB bugs and we culture the samples in a medium that the T. Bacillus likes. It’s quite a fussy thing, it’s quite difficult to grow. It’s not like ordinary bugs, it doesn’t grow in two or three or four days. And it can take 6 weeks to prove that a culture is negative. So again, very frustrating if you’re waiting for those results, because sometimes people come to me from the TB and infection clinics, and they’ve gone through months of investigations, because people were so worried they might have TB, and they’ve had to wait for all these culture results, and so on. So, it can be quite frustrating, but it just is really, really important.

There is a not terribly common cancer that some of you may have heard of called lymphoma, which also affects the glands, and that can cause, particularly in younger adults, it can cause sweats and fever and cough and malaise and fatigue, and enlarged glands in the chest, and so it can look a bit like sarcoidosis. And we really have to rule that out, and that’s why we like to have a biopsy if we possibly can, or we need to see that actually things are improving, maybe without treatment. But we don’t want to treat a form of cancer with steroids, because it will make the patient feel better, but it will delay the ultimate diagnosis. So those are two diagnoses we have to try and rule out if we possibly can.

Okay, so then we come on to treatment, and we have a bit of leisure with the lungs. Just to wind back, sarcoidosis can affect anywhere in the body, and it can affect, for instance, it can affect the heart. It can affect the brain, it can affect the kidneys, it can affect the skin, it can affect the joints. It doesn’t often affect the brain, the hearts, and the kidneys, but it can do. And in those situations, you really do have to start treatment immediately. They’re what are called ‘vital organs’ in medical jargon. And it’s very grievous to me that the lungs are not considered vital organs, because they are very vital.

But we do have a bit more time, and we can usually do a bit of watching and waiting, not always, but often we can. So, the things that we take into account when we’re thinking about treatment for lung sarcoidosis are your symptoms. So how bad are they? So, you know, if you come and you really can’t walk, and you can’t work, and you’re really breathless, and it’s really disabling, then we may have to think about early treatment. Or if your symptoms are getting worse, so maybe your breathlessness was quite mild to start with, and then it’s getting worse, so then we, you know, we really do have to take this quite seriously, particularly, as I say, if we’ve ruled out other causes of worsening breathlessness.

Now, the lung function, is really important, and if that is significantly impaired, again, or it’s getting worse, we think about treatment. And if the radiology is significantly abnormal, again, or getting worse, we also think about treatment. I have to say, and this is related to one of the questions we’ve received in advance, which is a very good question, the symptoms and the radiology, and the lung function do not always correlate. And we don’t really understand why that is, if I’m honest. There’s all sorts of hypotheses. But you can have quite bad radiology with virtually no symptoms, and very limited or normal lung function, or limited impairment. You can have horrible lung function and actually very not too much on the radiology. And symptoms can be discordant as well. And this is probably because other things as I say, can cause the symptoms. So, we have to put everything together, we have to look at all the other factors when we’re thinking about treatment, and it is worth considering all these things, and not just rushing in. And that can, I think, can be very frustrating for people, because they’ve waited for weeks, if not months, for a diagnosis. And then what they’d really like is a pill to make them feel better, which won’t have any side effects, and it’ll all be over in two weeks. And we’d all like that. And if I had that, then I would be doing very nicely, I think as a sarcoidosis specialist, but we don’t have that, and so we have to sort of go through all these things together.

And so it’s very much a discussion and a dialogue between the doctor and the patient, and this is emphasised, actually, in the 2020 British Thoracic Society Sarcoidosis Clinical Statement, and it’s a really good document, and it talks about how important it is to involve patients in the conversation. It’s also important, because of these reasons.

So why do we not just jump in and treat lung sarcoidosis?

Well, if you’ve got lung disease, but you’ve got those symptoms, it’s really difficult justifying giving you tablets, which might have side effects. Remember, all treatment has some side effects. It’s impossible to alter the body’s mechanisms, if you like, and not have some effect somewhere else. It may be very mild, it may not bother you, but there will be something. So if you’ve got those symptoms, we would have to think very carefully about whether to treat you. And the other thing is that, and I’ll come on to this, is that you may have self-limiting lung disease, very mild. Maybe very mild. So, I think I’ve come onto this on a later slide, but about a third of patients will actually get better without any treatment. So you really don’t want to give people treatments which can have side effects, with that, so you’ve got a time to watch and wait. And then there is this thing, again, I’m sorry, there’s a typo on the screen, it should be Löfgren syndrome, but this is a specific combination of symptoms, so fever, swollen glands, painful joints, and a skin rash. And that’s a very typical cluster of symptoms, and that often gets better on its own without any treatment. Now, it can be quite painful. So, you may need ibuprofen, for instance, for that, or Voltarol, and sometimes specialists will prescribe a very short course of steroids, but it’s usually two weeks or so. So that usually just gets better without any treatment, so we just wait, watch and see.

So, in terms of medications, some of you will be familiar with all these. So, steroids are the first line. So the examples are Prednisolone, that’s the commonest oral tablet form of steroid in this country. Methylprednisolate is intravenous, and then we have a few inhaled forms of steroid. And then there are a few other drugs that we commonly use, and I’ve listed them here. I’m going to come and talk about those a little bit more in a moment. So, I thought I’d go through the good points and the bad points of all of these just to summarise, and some of you may have had these discussions already with your specialist.

A good point about steroids is that we know them very well. They’ve been used since the 1950s, so we have a vast wealth of experience. And going on the basis that the devil we know is better than the devil you don’t, that’s a good thing. It doesn’t mean they’re necessarily the best, but we know what we’re dealing with, we know how to manage side effects. We know how to talk through the side effects with people. The other good thing about them is that they nearly always work. They nearly always make you feel better and they usually act pretty quickly. So, often when people first start steroids, they come back in 4 weeks, four, six weeks, and they say, oh, I feel wonderful, Doctor. It’s very nice, isn’t it, when that happens? It’s very nice for everyone. The doctor feels good and you feel good, and everybody’s happy.

But they have some very significant bad points, and the real bad points are all the side effects. They have short-term side effects, and they have longer-term side effects. They can cause an increase in appetite and weight gain, and that can be quite significant if people aren’t able to manage it, because it can make you feel really, really hungry. And it can be very, very difficult. And sometimes people come out and they’ve put a stone on in a few weeks, and then they feel breathless, and they’re feeling breathless because they’ve put on so much weight, and it’s really difficult. I’m very sympathetic to that, so that’s a real problem.

They can, if you’ve got diabetes, they’re bad news, because they will push your sugar levels up, and then you’ll need more treatment, and that may be difficult. They can put your blood pressure up, they can thin the bones, they can make you more prone to infection, they can cause bruising and thinning of the skin. Longer term, they can predispose to cataracts and glaucoma. So, there’s a whole host of problems with steroids, which means that if we use them, we like to use them for as little time as possible, in the lowest dose possible. So what we tend to do is we tend to start on a high dose, get you feeling well, and then we gradually bring it down, and that’s probably a trajectory that a lot of you are familiar with.

So, another alternative way of giving corticosteroids is an infusion, and this is done intravenously, and this is intravenous methylprednisolone. Why is that quite good? Well, you don’t have to worry about taking tablets every day, so that can be quite nice. And this… there’s a suggestion… well, it sort of shortens the duration of the side effects, because you get side effects, but they’re sort of limited to the day that you have the infusion. Very often, what we do is we give three consecutive days of quite a high dose as a day case. If you’re diabetic, for instance, you can be monitored during that admission, so it provides some reassurance to you, and the doctors and the nurses about your diabetes, for instance, and we can monitor for any other side effects.

But the bad points is that we’re giving a very big dose, and it can have profound side effects, particularly on the bones, and so we usually try and measure bone density simultaneously at the same time. I mean, not during the infusion, but around the time you’re starting treatment. We do that with a special x-ray, which takes about 20 minutes. So, we do use them, but we don’t do them too often, and we would have to have a very good reason to treat, I think, with intravenous steroids.

What about inhalers? So we use steroid inhalers, as some of you know, in asthma, and they’re really good, they’re really effective. And the really good thing about storage inhalers is that they have limited side effects, because they target the drug to the airways. Now, one of the things I didn’t talk about when I talked about lung function was that you can get different patterns on the lung function as well. I talked about the fact the lung function could be discordant and not related to symptoms and radiology. But I didn’t say you can get different patterns.

So you can get an airways type disorder that mimics asthma with cough and wheezing. And steroid inhalers may be useful for that, possibly, but they’re a bit controversial, because there’s not an awful lot of evidence that they’re effective, and they’re not actually routinely recommended. So they’re not in the guidelines. But I have to say, pragmatically, they’re sometimes useful if somebody’s got a cough. But there are lots of other causes of cough as well, and we’ll come on to that later. So, sometimes used. Not officially recommended but sometimes people find them of benefit.

So those are the steroids. Now, we come on to a host of other drugs which are classified as immunosuppressants, so they suppress the immune system. And the commonest one that’s been used for quite a long time is methotrexate, and it’s always been the first line, second agent. But there was some quite exciting data released in May of this year, the PREDMETH study, which sounds much more exciting than it actually is, but anyway. This was comparing, prednisolone with methotrexate. Let me just clarify that it showed that methotrexate actually was as good as steroids over 26 weeks, and it looked as though the effect wasn’t as quick in onset. So if you remember, I said that corticosteroids are quite good, because they act really, they generally act well, and they’re effective, and they act quickly.

So you’ve been waiting and waiting and waiting for your diagnosis, you feel absolutely awful, and suddenly, suddenly, because you’re tired, you feel wonderful. That is great. Now, methotrexate doesn’t do that. It takes longer to kick in. But it does, at 26 weeks, look as though it’s actually non-inferior to steroids, and there was a suggestion from the data that the benefits might continue to increase after that period, while the effects of steroids might have plateaued.

So, some really interesting data, and I think it’s quite encouraging, you know, if you have somebody who’s got relative contraindications to steroids, for instance, somebody with diabetes or somebody who’s overweight, then. It could be an alternative. So that is quite encouraging.

Another advantage is thatyou only have to take it once a week. That could be a disadvantage, of course. You’ve got to remember which day to take it, so we always ask patients to choose a day that’s memorable for them, might be a Sunday, might be a Friday, might be a Saturday, might be a Wednesday, but they need to remember to take it once a week. It’s not a daily tablet. And they have to take a vitamin tablet, folic acid tablet on the day after, usually, once a week.

Really bad points: it’s teratogenic, which means you can’t take it and get pregnant. And that affects the male partner as well, and you have to be off it for 6 months before you try to conceive. So that’s really significant for anybody who’s thinking about starting a family, and you need to have really good counselling about that. It needs regular blood monitoring, because it can affect the full blood count, affect the liver, and so usually you would need bloods every two to four weeks, starting off in the early stages, and then you would, once you’re stable on a stable dose, because you might start with a lower dose and increase, you may just need bloods every 12 weeks, and some general practitioners will prescribe this and monitor it. But they’re not obliged to under current commissioning guidelines. They will only take it on if they’ve got the resources and they feel competent to do it. So you may be limited to coming back to your specialist for the bloods and the prescription.

It can make people feel unwell on the day that they take it. So, I sometimes say to people, we might want to take it on a weekend day, or a day where you’re not going out and working, and you’re not going to have to be at your best, and you can rest a bit at home. Doesn’t always. Some people have no problems with it at all, but some people feel unwell. And because it’s an immunosuppressant, it increases the risk of infection. So, good points and bad points for methotrexate.

What about hydroxychloroquine? Hydroxychloroquine, again, good because it avoids the side effects of steroids. It can be useful in my experience, for fatigue and joint pains, and skin rashes. It’s generally well tolerated. It’s considered safe in pregnancy, which is a plus, and it’s probably not as strongly immunosuppressive as some of the other agents.

I always do a particular blood test called a G6PD before starting. That is recommended, because there are a few people in the population who don’t have enough of this particular enzyme, and it means they can’t break down hydroxychloroquine, and it can cause damage, and it can be, um, it can cause haemolysis, damage to the red cells. So it’s really important to do that blood test. Again, it needs regular blood monitoring. It can make people feel sick, and it can give them indigestion. Not everybody gets that at all. I mean, I have lots of people who take hydroxychloroquine who find it very useful, but, you know, it can cause those side effects. And then it’s related to chloroquine. Which is used as an anti-malarial. And chloroquine can damage the back of the eye. Now, hydroxychloroquine doesn’t do it, anything like as often, and that’s why it’s used preferentially in sarcoidosis, as opposed to chloroquine. But it can upset vision, and particularly sometimes colour vision. And if you’ve got pre-existing eye disease, you’re going to have to get your optician or your ophthalmologist to approve it, because they may say no. So those are things just to think about with hydroxychloroquine.

Now there’s another one called Mycophenolate, this is another immunosuppressant. Again, great, because you might be able to avoid steroid side effects or reduce the dose of steroid down to the point where you haven’t got so many steroid side effects. Usually well tolerated, it’s used originally in transplant patients, particularly renal transplant patients and can be effective. Bad points, well, again, it’s teratogenic. So you can’t take it if you want to start a family. There’s really rather limited evidence for its efficacy. It needs regular blood monitoring. And it’s an immunosuppressant, so it increases the risk of infection. You get the picture here. They’re all rather similar.

What about Infliximab? So, Infliximab inhibits TNF. TNF is a cytokine, a small protein that’s elevated in sarcoidosis. It’s also elevated in another number of other inflammatory conditions and it was originally introduced as a treatment for rheumatoid arthritis many years ago now. It can be effective where other drugs have failed, and we can use it in people who don’t have lung sarcoidosis, but have troublesome, what we call, extrapulmonary sarcoidosis, and it can be really useful. You have to have it by injection or infusion. There is some evidence for efficacy in lung disease, so I think if you had somebody with lung disease and you had nothing else, you tried everything else, there would be a case for trying to use it, but there’s limited evidence for the benefit in lung disease, and the benefit’s probably quite small. You need very thorough pre-treatment screening. You need regular blood monitoring, and it is strongly immunosuppressant and increases the risk of infection, so again, we’re quite cautious with Infliximab, although we do have it in our set of tools.

So, when we start medication, what are we looking for? As I’ve implied earlier, we’re not likely to get an instant cure. Sarcoidosis doesn’t usually go away that quickly. If it’s got to the stage where your specialist is talking about treatment, it’s likely that you’re going to need treatment for some time. But we want to see an improvement in symptoms. We would like, if the lung function is impaired, to see an improvement in lung function, and we’d also like to see an improvement in your imaging. But of course, for you, probably the symptoms are the most important. And we have to worry a little bit more as sarcoid specialists about end organ damage, so lung function, and the radiology.

Now, the million dollar question is, how long will you need treatment for? And this is a very good question, and we can’t really answer that very precisely. Now, one of the things we’d really, really like is we would really like the ability at diagnosis to say, you fall into a group where the group will not need treatment, and the disease will get better on its own. That’s around a third of patients. Or you’re going to be in around a third of patients who need treatment for 3 to 5 years. Or you’re in that group that are going to need longer treatment. And we don’t have a specific predictor. We do have clues. I did say that diagnosing and managing sarcoidosis is a bit like being a detective, so we do have clues. I talked about Löfgren’s syndrome and the fact that gets better, generally, on its own without treatment. That’s a classic picture of sarcoidosis. So people with Stage 1 sarcoidosis, are quite likely to resolve without treatment. So if they’re well, we may not, as I say, we may not need to treat them. But I would say usually when people ask me, I usually say you will need treatment for at least 3 to 6 months. Obviously, if people don’t tolerate treatment, then we have to review and think about something different. So you wouldn’t put somebody on treatment for 6 months and not review them, and not check that they’re all right because they may be having side effects, and they may find the treatment very difficult, and we may need to adjust the dose. But you’re probably going to need it for at least 12, if not 26 weeks, just to see what the benefit is, and repeat all the tests. And I think that can be disappointing for people for completely understandable reasons.

Again, the complexity of making the diagnosis, the complexity of predicting disease course, the complexity of management, can make it feel as though it’s all a bit difficult, and it would be lovely to have something that would just get rid of this inflammation quickly and on its own with no side effects. But that’s the ultimate.

goal, I think. And then that’s, I suppose, just before the goal that you heard earlier, which is to make sure that nobody gets the disease in the first place. And that’s a little bit further ahead. That is the ultimate goal.

So, there was a question I had about other treatments. Steroids are the main treatment options for sarcoidosis, is that really the only option? What else can patients do to help manage symptoms and improve lung function? So there are a lot of other things that your doctor and your nurse may talk to you about. All right, these aren’t in any particular order. Pulmonary rehabilitation, a bit like cardiac rehabilitation, which was sort of piloted and started really originally in the 70s and the 80s for people after heart attacks. So, this is a really good programme. It’s offered on the NHS on a regional basis, and so you go somewhere local, like a local gym and you’re offered in a group 2 or 3 times a week for up to 6 weeks, a programme of exercises which will be tailored to you and your ability, and you’re given general lifestyle advice. And then some of these schemes then have a system where you can then join the gym at a discount or carry on with that programme or you’d be given a set of exercises to follow at home. And the reason this is good, and again, it relates to another exercise, another question you asked about, was asked about, can you improve your lung capacity if you have pulmonary sarcoidosis through exercise? No, you can’t really improve your lung capacity, but what you can do is you can improve your general health and your muscle strength. And you may remember I said at the beginning that one cause of breathlessness is deconditioning, or just being unfit.

And there’s always a little bit more you can do just to strengthen your muscles, just to make sure that your muscles are functioning as effectively as they can. And that will help with breathlessness, so it doesn’t really increase lung capacity, but it does help with general fitness, and that’s really the premise of pulmonary rehabilitation. So that’s important. There is a caveat, though. If you are already very fit, they won’t take you on. So if you’re playing golf three times a week, and you’re doing 18 holes, and you’ve got a really good low handicap, they probably won’t be very interested. So we can only refer people if they’re already quite disabled by breathlessness.

Sometimes, I mean, typically people have a dry cough. But sometimes they, if they have scarring of the lungs, they can have recurrent infections, and they can get sticky, thick phlegm, and it can be very difficult to cough up and very exhausting, and so we can give medication to make that phlegm looser. We also recommend that people, if they have chest infections, so bacterial infections with yellow or green phlegm, they get antibiotics promptly from their doctor, preferably with a sputum sample sent for culture first, so we know what we’re treating. But it’s really important not to ignore those symptoms, because that can lead to further cycles of infection and lung damage.

It’s really important not to smoke. I mentioned that earlier. There’s no evidence that smoking is linked to sarcoidosis, unlike, for instance, COPD or lung cancer. But there’s no doubt, if you smoke, you are inhaling  agents that are damaging your airways, damaging your lungs, and you’ve already got inflammation and damage to your lungs, and the last thing you want to do is to make that worse. So you really do need to get help with stopping smoking. It isn’t straightforward. It can be very addictive, we know that, but there are lots of strategies now to help people with smoking cessation, so go and talk to your practice nurse about that, or your pharmacist. There’s lots and lots of different things now that can be prescribed.

I mentioned cough, and I said that cough has a lot of other causes other than sarcoidosis. So, for instance, you can have acid reflux. And that can be what we call silent acid reflux, so you don’t always get symptoms. But indigestion, heartburn, that can cause a cough. You may have coexistent asthma, because asthma’s terribly common, and that needs to be treated. You may have hay fever, that can be associated with a cough. You may have a post-nasal drip, so you may feel it starts dripping down the back of your nose into your throat. That will cause a recurrent cough. Or, more severe sinus disease. So all those things will be looked at during the course of your management, just to see whether there’s anything else we can do to treat cough, because those things can be treated and are fairly straightforwardly. Of course, smoking is a great cause of cough, so another reason to stop smoking.

Relaxation breathing techniques. There’s no doubt if you have a lung condition, it’s very tricky sometimes to get your breathing into a regular pattern afterwards, if you’ve had a severe infection, or you’ve been really unwell. It’s really difficult, and we recognise this increasingly now, and there are some really good exercises. There’s lots of resources that you can use, there’s information on the internet, there’s a dysfunctional breathing pattern website, which is really helpful. There are apps, mindfulness apps that can also focus on breathing and just help people to really get the benefit of those and help manage their breathlessness.

Sometimes we have to think about home oxygen. So I talked about the 6-minute walk test, and I talked about people dropping their oxygen levels below 90%, and at that point, we would offer an assessment for portable oxygen. People don’t always want portable oxygen because they have to carry it or pull it behind them. And it’s quite tedious, but sometimes it makes a big difference to quality of life, because it means you can get out and about, or you can go away and travel, and you’ve got much more freedom. So that is always an option, if people need it, but it is only prescribed to people who need it because oxygen, for people who have normal oxygen levels can be dangerous and toxic, so it’s not a remedy in itself. This is really just to support people who have got low oxygen levels.

And then sometimes we refer to community palliative care, not very often, but those teams are really good at managing symptoms, so just sometimes we have people with an intractable cough, but actually they’re otherwise really doing pretty well and the community managed care teams can prescribe a very low-dose morphine, and provide some support with cough or breathlessness. And so sometimes those teams have a role, and it doesn’t mean that we’ve stopped treating the sarcoidosis, or we’re going to stop seeing you in hospital, necessarily it just means that we’re getting some extra support for you at home with symptoms.

Right. Okay, now I’m going to deal now with, a relatively uncommon complication, but some of you may have come across this complication called pulmonary hypertension. It’s abbreviated to PH, and this is distinct from central systemic hypertension, which is the raised blood pressure that you’re all familiar with, and everybody’s familiar with. So when you go to your doctor, or your nurse, or the pharmacist, and they measure your blood pressure, they’re not measuring. pulmonary hypertension, they’re measuring systemic hypertension, they’re measuring blood pressure on the left side of the heart, okay? But this is raised blood pressure affecting the arteries in the lungs on the right side of the heart. And sarcoidosis can cause this. And it can do it in one of two ways. Quite rarely, but it is recognised, it can directly cause it through inflammation of the blood vessels, or even destruction of the blood vessels in the lungs themselves. And that can be quite difficult to pick up.

It’s not necessarily always obvious on standard imaging or lung function, so you might want to have to suspect it, think about it, and it can also indirectly cause pulmonary hypertension if there’s lung scarring, because that can cause low oxygen levels in the blood. Low oxygen levels in the blood can trigger a rise in blood pressure on the right side of the heart. So there’s two different mechanisms. As I say, it’s not terribly common. But if that is the case, you will probably be referred to a pulmonary hypertension specialist, and these can either be lung specialists who specialise in this area or they can be cardiologists who specialise in this area, and so you may end up having your care under a couple of different teams, even though you’ve only got the lungs involved, you thought.

All right, I thought I’d mention lung transplantation. This is going to be a very small minority of people with sarcoidosis. Most people we can treat and treat effectively and, you know, we can get a good quality of life and people feeling well on treatment, and we can manage the side effects of treatment. There are a very small minority of patients where they get worse despite medical treatment. And worsening breathlessness, worsening lung function, increasing oxygen requirements, and at that point, we might be thinking about referral to a specialist lung transplant centre. Now, in this country, it’s not considered for anybody over the age of 65, and that is not because of any prejudice against people who are over 65, it is merely that the data show that people over 65 do really badly with lung transplantation. It’s major surgery, as you can imagine, and what you don’t want to do is to transplant somebody and have that transplant fail. So you want to maximize the chances of a good outcome.

There are some absolute contraindications. They include active malignancy or cancer. They include obesity and they include the inability to get the prednisolone dose, to 10 milligrams once daily. So 10 milligrams once daily is fine, but higher doses impair wound healing. So again, bad news if you’re having major surgery. And so that’s the contraindication.

And then other comorbidities, technical term for other medical conditions you may have, are relative contraindications, and those would be considered on a case-by-case basis. Okay.

Now, there was a question about air travel. And so, because I’ve chaired the BTS air travel group for a number of years, I thought I should probably just mention a little bit about air travel, because most people like to fly at least once a year to go overseas and have a holiday. It’s generally safe in people with lung disease. There aren’t that many people who have problems. However, you just need to be aware when you fly, your aircraft will be pressurized to 8,000 feet. At the moment, if you’re in London, like me, you’re at sea level. So you’re not at 8,000 feet. And when you go to 8,000 feet, your oxygen levels will fall, and even a healthy person’s oxygen levels will fall. But if you’ve got low oxygen levels to start with, then it will fall further, and that’s where it could be a problem. So, low oxygen levels are just one of the things that we have at the back of our minds when you’re thinking about flying. You’re often not very mobile on a long-haul flight, so you may sit in a seat and not move around very much for several hours. That is not, of course, specific to air travel, it could also happen on a long train journey or particularly long coach journey, where you just sit in your seat and you don’t move for hours, and that can predispose to clots in the calf, or even clots in the lung. And there is a suggestion that actually the altitude exposure also makes the blood a bit stickier, and so that can also predispose you. We do also think about your risk of clots when you’re traveling. And then just the really obvious thing, that if, you’re traveling in economy, like most of us most of the time you’re at increased risk of infection. Now, people worry terribly about the risk of infection on aircraft. The filters they have in aircraft cabins are extremely high quality. They are the same quality as are used in hospital isolation rooms, so they are really, really good. So you’re not going to get infection from somebody who’s a long way away. But if you are sitting in a crowded cabin, and you’re sitting right next to, or very close to somebody with a streaming head cold then the chances are you may well catch it. And that’s just common sense, I think, rather than getting too excited about the risks of infection on aircraft, and there’s not much you can do about that, and that’s been shown, for instance, with TB, and the reason we know that with TB is that TB is obviously a notifiable disease, and we do contact tracing for cases of TB, and we’ve shown that if you sit close to somebody on an aircraft with TB, then you’re at high risk of it getting infected and TB, open pulmonary TB, where you’re infectious, is an absolute contraindication to air travel. And that’s WHO guidelines.

Now, I’m going to show you two algorithms very briefly that we use now, and we published in our 2022 clinical statement on air travel.  I’m not going to go through them in any detail, and I know the screen is quite small, but these are the algorithms that we advise healthcare teams to follow. So there is one for airways disease. So do you remember I said that sarcoid could cause an asthma-type picture? So, for airways disease, we need to know things like what are your oxygen levels at rest on air? What is your breathlessness score, your MRC Dyspnoea score, which I talked about earlier? Are you already on oxygen? And so on, and then we may need to do a walk test, and then from that, we can think about, would we recommend in-flight oxygen, or would we recommend further testing, which is this hypoxic challenge test, or HCT. So it’s a bit technical, but there are things that we can do to help with that.

If you have scarring of the lungs, you have a rather different picture on your physiology, and you react rather differently to low oxygen levels than people with airways disease. There’s quite a lot of evidence supporting that now, which is why we have two different pathways.

So this is the pathway for people with scarring of the lungs. And we would normally want a 6-minute walk test or another exercise test. We might want to know about your oxygen levels and your diffusion to see how well you’re going to manage with air travel, and we would either recommend that you should be fine or you might need to have oxygen on the flight, or we might want to recommend further testing. So those are the things that we consider when we’re thinking about air travel.

Okay, now, some useful resources, and I haven’t listed them here, you’ve got a wonderful resource from SarcoidosisUK. I don’t need to tell you that, that’s why you’re all here, so I’m preaching to the converted, and I think the SarcoidosisUK team do a fantastic job. So there’s lots and lots of information there. There’s really good information on air travel and lung disease that Asthma & Lung UK, which is our, our major general lung charity in this country. And if you want to know about airlines and oxygen, the European Lung Foundation, and Graham, if you want me to circulate the links later, I will, the European Lung Foundation has a list of all the different airlines, which it updates, and what they charge for oxygen, and what sort of things you have to do before you travel with them. So those sorts of things are really, really useful. And then apps are good, for mindfulness and breathing pattern disorders. And as I mentioned, there is the breathing pattern disorder website.

Okay, so I’m coming to the end now of my slides. I’m going to come on to questions, and I think what I’m going to do I’m going to stop sharing my screen.

Hopefully you can see me now. Well, you may not want to see me, but you can see me. I think we’ve covered a lot of the questions. We’ve talked about oxygen diffusion, lung capacity, exercise, risk of flying. There was a question about fatigue being a factor for sarcoid, other exercises that can be done without increasing other risks. I think the pulmonary rehab is the answer for that. There is, of course, an awful lot of debate about fatigue in sarcoidosis and other conditions, and where it’s disabling fatigue, I think that’s quite tricky.

I was hoping and I think we may still see some fruit, that the fatigue that was associated with COVID would lead to more research into fatigue. I mean, it has led to some research. I’m not sure we’ve got any obvious answers. There are medications we can give, they’re not licensed in this country, so it’s quite tricky. And I normally go through lifestyle measures. And so on, but I think gentle exercises are always good just to try and keep yourself as fit as you can.

Graham: Actually, while you’re talking of pulmonary rehab there, we’ve had a question that’s actually come in while we’ve been talking here asking whether it can be used if you also have long COVID and PEM.

Robina: Yeah, sure. Well, it’s a really good question, Graham. So, the long COVID, there’s a lot of information on the NHS website, and physio websites about long COVID. If you’ve been lucky enough to, fortunate enough, to be referred to a long COVID clinic they have had access to rehab programmes which are probably not dissimilar to pulmonary rehab, but possibly a bit more tailored to people with very severe fatigue. So those are options. The other thing that is interesting, of course, is the whole thing around singing for breathing, which is a more general thing, but can really improve wellbeing, and, you know, it uses the respiratory muscles.

So, although it’s not involved, you know, you’re not having to walk, you’re not having to use weights, you’re not having to do things that might make your general fatigue even more disabling, might actually help you with other things, so that’s also something to consider, and you can talk to your specialist about that.

I’ve got other questions I haven’t answered, Graham, so I’m happy to go on to those unless you interrupt me, but I’m happy to be interrupted.

Graham: One of the questions you’ve got there relating, obviously, to cancerous nodules has also been asked, actually during the session today as well, so you could cover that one maybe?

Robina: Yes, yes. Yeah, sure. So, there is some data suggesting that sarcoidosis has a possible association with some cancers. It’s not a direct link. We don’t regard sarcoidosis as a precancerous condition, it’s not a malignant condition in any way and those tumours can be things like lymphoma and haemophilical cancers, but as I say, it’s not a precancerous condition, and this is big data suggesting some possible associations, so it’s tricky. Now, in terms of pulmonary sarcoid and nodules in the lung, we know that lung scarring of any cause does predispose to lung cancer. So we always just have that at the back of our minds. But the other thing, of course, that happens, and I did allude to this, is that high-resolution CT scans now are so sensitive and so good that we endlessly pick up nodules that we don’t know what to do with, and there are now very well-established British and international and European guidelines on managing lung nodules.

So you may have sarcoidosis, and there may be a lung nodule there that nobody’s quite sure whether it’s a gland, an intraparotid lymph node, and it’s of no significance. The only way to tell usually is to do serial CT and follow it up and so you may find yourself in this loop of having to have repeat CTs. And, you know, that can be quite worrying for people, and it’s really tricky, that, and we see even more of it now with low-dose CT screening for lung cancer, so we’re picking up lots of nodules.

There was a question about, is it likely to come back? Was the lung nodule likely to come back? Well. Yes, I mean, it depends on the treatment for the lung nodule, isn’t it? If it’s a cancer –  I’m not a cancer specialist. What I would say is usually the risk of a nodule being malignant is calculated using something called a Brock Score which is done by MDT’s specialised in lung cancer. And that plugs in risk factors for lung cancer, and then that helps you decide whether that person is more at risk of lung cancer than not. And I guess, as a non-cancer specialist, so, I’m not sure I want to be quoted on this without reference to my lung cancer colleagues, but as a non-cancer specialist, I would say the risk is likely to be related to your individual risks for lung cancer. Rather than your sarcoidosis. And I hope that helps.

There’s another question, which is a very good one, about symptoms, if there’s no evidence of sarcoid being active. Can you continue to have symptoms if there’s no evidence of sarcoidosis being active?

Really tricky, that one. I think I’ve shown you that there are a lot of other potential causes for symptoms. Which aren’t necessarily related to sarcoidosis, so it’s really, really important to look at those and make sure there’s nothing else that’s easily treatable that is causing your symptoms. But there is no doubt that with the tests that we have, as I say, we don’t have a specific diagnostic test, we don’t have a specific test for monitoring, I can’t just bring you back every year for a blood test and say, yes, it’s cleared, or it’s still active. So I think it probably is possible for people to still have symptoms when we can’t find an awful lot of evidence of activity. But of course, this is really difficult for doctors to deal with because the patient is suffering the symptoms, and the doctor can’t see anything on the tests. And that is a real problem, and it’s a problem because, you know, the first premise of medicine is to do no harm. And what I don’t want to do is to poison you with a tablet that’s going to cause harm, even if it’s in 20 years’ time. So that’s why doctors are so cautious about giving medication if they can’t see any abnormalities. And that’s why there may be quite a bit of dialogue around that question, and so I always say, look, if we can’t see anything on the test, it’s good news, because it means we think your lungs and your heart and everything are functioning well.

You still feel rough. What can we do? It might be that you have hydroxychloroquine for fatigue, it might be that actually you do something like singing for breathing, or we, you know, we think about other lifestyle measures, or is there another symptom behind your cough? Do you actually have asthma? We need to treat that. So, it’s taking that sort of holistic approach, I think, is actually really important, but I appreciate it can be really frustrating for patients. And it is very difficult in the course of the usual, NHS hospital specialist clinic, and the time that’s allocated. to do this well. So, at the moment, you know, I often have the luxury of up to 30 minutes with a patient, but that has to include all the, you know, include the admin time. But that is being cut, you know, rapidly to 20, and sometimes I only have 15 minutes. And that’s got to include, you know, calling the patient in, sitting down, looking at the records, etc. So, it’s difficult, you know, and some of my colleagues are under more pressure, than we are here at Imperial, so it is tricky. And I think that’s why, you know, a charity like SarcoidosisUK is so helpful, because you can provide that sort of backup and background support.

There was another question about diet for pulmonary sarcoidosis. A patient reports that breathing is definitely better without too much carbs and sugar.

I’m not a dietitian, and, I would hate to say anything that was taken to be official. However, for many years, I have thought that the Western diet is very heavy and exceedingly heavy in carbohydrates and processed foods, and sugar. I think we have demonized fat over the years. And that has meant that everybody’s been pushed onto low-fat diets. But if you look at low-fat foods, they always contain more sugar than the high-fat variety. So, I think for patients for whom it’s helpful, or patients for whom controlling their weight is a problem reducing the carbs and cutting out sugar can only be a good thing. But not everybody will find that helpful, or need to do that. So, I think it is a conversation I do have with my patients very often. It is tricky, because dietary advice is controversial. But I think even the American guidelines now are swinging away from demonising fat and pointing out that actually you do need healthy fat, and it’s the constant avalanche of sugar and refined foods and refined carbohydrates that are the problem you know, a Western diet, make it actually very difficult for people to control their weight as they get older. It’s not a problem when you’re young, but it is definitely a problem as people get older.

We’ve got a question about Infliximab and chest pain and breathlessness. Would you like me to take that now, Graham? Would that be helpful?

Graham: Yes, yes, go for it.

Robina: So I got a question you gave me. A patient responding well with Infliximab, so that’s good. Asks if chest pain and breathes mean the damage could be deemed done for the lungs.

So, Infliximab can very rarely adversely affect the lungs. That is a recognised problem with Infliximab, but it is rare. And you will be, if you’re taking Infliximab, you will be monitored for that. So I would be very surprised if your specialist didn’t pick up if there was any damage to the lungs. So I would hope it didn’t mean that. Chest pain is, as I alluded to earlier, is a really difficult symptom. We don’t really understand the mechanism of chest pain, I think, in sarcoidosis. I think we now have, well, I’m sure we now have a much lower threshold for suspecting cardiac sarcoidosis, so that’s heart involvement by sarcoid. That’s a bit out with this… my theme, but you know, I think if you do have really troublesome chest pain, then it may be that it’s worth screening for, damage to the heart.

The only one I haven’t covered, which is very general, Graham, you may want to push this into the general Q&A, I don’t know, is it common for people with pulmonary sarcoid to have pressure on the lungs from the stomach or the abdomen? This patient experiences bloating and water retention and feels there could be a connection. I mean, it’s a good question, and if you’ve got that, it’s very difficult to deal with, and I’m very sympathetic. Of course, it’s quite a non-specific symptom, so it’s quite difficult. I don’t think it’s related to lung disease. Sarcoid can affect anywhere. And it can affect the gastrointestinal system as well, so it’s worth just being aware of that, but what we don’t want to do when we see people is just to do endless investigations that are actually really quite invasive, quite unpleasant and might show a minor abnormality, which then causes all sorts of concern, which may not be related to symptoms. It’s really, really tricky. The other thing I would say is, of course, that bloating and fluid retention is a side effect of steroids. And this is the other problem with non-specific symptoms, is that they can be a result of the treatment. And that’s why it’s really important to have your condition managed in a clinic that does this quite a lot, because there’s no doubt the more experience you get with these therapeutic agents, you know, the easier it is to try and manage the symptoms and give appropriate advice. So, I think, you know, that is tricky. And there could be lots of things that are causing that.

Graham: Well, thank you. I actually was joking with the office team that if we had technical problems or people ran short, I might sing a song, and now you’re telling me it’s NHS advice!! So, don’t worry, I’m not going to, though.

Robina: Absolutely. Singing for breathing. Singing for breathing, yeah.

Graham: I’m definitely not going to, but we will include John Scadding, I think, as an answer in our next quiz night.

Robina: Absolutely. I think we should, yes. 

Graham: Because we really should all know about him, shouldn’t we? No, thank you very much for that. A particular takeaway for me was seeing all those treatments side by side as comparisons, because we don’t tend to see that, and that was very, very helpful. But I think also a takeaway, which we’ll take away with us the office, in due course is, you’re talking about language. Now, we’re all used to seeing medical language, and as you say, the archaic stuff that’s used. And I think maybe a simple glossary needs to be done on our website, how to understand your consultant in plain English, because a lot of people, I think, do see a whole string of semi-Latin words, and that must be something terrible, mustn’t it? When, as you said, it was based on cheese, one of them, wasn’t it? So we’ll pick that up, I think, and we’ll do that as a future change.

Thank you again for your time there. It was an epic session, so really, really good.