QD clinics on ILD - lessons from the Clinic #4 Save

Hi, everyone. This is Aurelie Naj from Glasgow. And today we are on for the QD clinic. I'm going to talk to you about a patient that has been attending my clinic for about four years now and the situation we have been on over time. So this lady is 65 years old and she started attending the rheumatology clinics four years ago for originally issues with coughing and breathlessness, and that led onto diagnosis of diffuse systemic sclerosis.

So at the time, the lungs were crackling, there was some skin involvement. And actually upon asking her how long she's been struggling with her cough, we also realized she had a Rheum's for about ten years, and then she had obviously telangiectasia, and then the antibody profile was RNA polymerase III antibodies and anti ROT52. And so that at the time told us that, as you know, she was more likely to have severe skin progression, rapid lung progression, as well as a higher risk of renal involvement. And so following that, she was rapidly diagnosed upon HRCT with a non specific interstitial pneumonia, as well as that was moderate under the CT at the time, and she was started on mycophenolate, as well as a small dose of glucocorticoids, because obviously due to the risk of renal crisis, we couldn't really get her on a higher dose. And so we kept seeing her over the years and she was doing pretty well until two years ago when things started to She presented again with worsening of her breastlessness, and on the PFT, we realized that the FCV had dropped about ten percent, so from eighty five to seventy five, and the HSCT confirmed that there was a mild worsening of the lung disease, and therefore we got her started on anti fibrotic.

And by the way, this combination of mycophenolate and indatinib with low dose of glucocorticoids is currently what's recommended for CTD ILD in the ESRUlar recommendation for CTD ILDs. Obviously, at the time they weren't published, but if you're interested to read more about these recommendations, there's a really nice article that's on the RheumNow website for the ILD month, so I invite you to go and have a look at that. But that's how we treated them, treated her a couple of years ago, and then things kind of stabilized for a while. Now, six months ago, she had lung infection, following what really, she didn't recover really nicely. She started to really complain of breathlessness to a point that we thought, okay, we're gonna have to do PFTs and HRCT again.

However, something in the PFT really rang a bell for us at the time, which was that the DLCO had dropped massively, so from fifty to nineteen percent with an FCV that was still roughly around seventy five percent. And so that for us was already a sign that something was not right, but not necessarily in the sense of her lung disease progressing, but maybe that was a sign for pulmonary hypertension. And so she got a transthoracic echocardiography, however, this is not always really helpful in people with systemic sclerosis ILD because of the lung fibrosis and the skin stiffness. The results cannot be always in favor of pulmonary hypertension, and so the recommendation in these patients with an unexplained worsening of restlessness or high risk of pulmonary hypertension, or when they're candidate for transplantation, the recommendation is to go to the right heart catheterization, which this patient had, although the echocardiography was already showing signs of moderate right ventricular dysfunction, which were obviously evocative of pulmonary hypertension. And so the pulmonary hypertension was confirmed in these patients, and then she was started quite rapidly with a combination therapy.

So in her case, it's always a bit more difficult. So pulmonary hypertension, when it's idiopathic or in systemic sclerosis without lung disease, usually the recommendation is to follow the guidelines for PAH group number one, because that's with our ILD. However, in her case, due to moderate interstitial lung disease, we know from clinical trials in this group, so the group three of patients developing pulmonary hypertension following prolonged hypoxia, the treatments for pulmonary hypertension don't work as well, and there's been quite a lot of negative trials in particular in severe ILD. In her case, we were lucky enough that the lung disease was not that severe. And so she was started on phosphodiesterase five inhibitors and then prostacyclin agonist as a combination.

And that helped a little bit for her symptoms because at that time the restlessness was so severe that she couldn't do any form of activity, even speaking was, as well as she needed oxygen. And obviously she's now on the list for transplantation as well. But I think one of the reasons I really wanted to talk to you about this patient is because we tend often in people with non interstitial lung disease to, in case of worsening of restlessness, to think about infection or to think about worsening of the lung disease. And I think it's really important in these people to remember as a learning point today that pulmonary hypertension is one to look for, in particular, when the restlessness can't be explained by the lung disease when the DLCO drops massively compared to the rest of the PFT parameters, and or when there is not necessarily no cough or no other signs associated with the breathlessness. And so for this patient, we're able to quickly diagnose her and get her on a treatment.

But that's definitely something to remember. On the other hand, I wrote an article recently about treatment of pulmonary hypertension in the context of CTD, I invited you also to look at that on the RheumNow website. So that's me for today. You're welcome to go on the RheumNow website for more QD clinic and enjoy the ILD content over the whole month of September for the campaign. See you later.

Welcome to ILDRA QD clinics. I'm Doctor. Brian England from the University of Nebraska in Omaha, Nebraska. Today's case is entitled Be Careful What You Look For. Now our patient today is a 57 year old female with a history of right knee pain and a diagnosis of osteoarthritis.

But she started developing hip, hand, ankle, and foot pain over the last few months. This is accompanied by about one to two hours of morning stiffness. She's a former smoker, have one pack per day for fifteen years, but quit eight years ago. Today in clinic, she has a BMI of forty three kilograms or meter squared. She has a little fullness in her MCPs.

Her lungs are clear to auscultation. Her serologies return with a CCP greater than 300. Her rheumatoid factor is 440, and her CRP is 2.7 milligrams per deciliter. Radiographs of her hand show possible MCP erosions, and the patient started on methotrexate, which is escalated up to twenty milligrams by mouth weekly. Within a few months, the patient has really complete resolution of her articular symptoms.

There's no synovitis detected on exam, and she's functioning well in life and work. She's doing so well, she decides that she's gonna participate in a research study where she undergoes a chest CT and pulmonary function test. The chest CT shows mild ground glass attenuation in the bilateral lung basis on both supine and prone imaging. And pulmonary function tests show an FVC of seventy seven percent predicted and FEV one of eighty two percent predicted. As part of that visit, she has detailed assessment of her respiratory symptoms, and she does admit to a bit of shortness of breath and dyspnea over of exertion over the past few weeks.

Now based on these findings, this case actually gets reviewed at a multidisciplinary discussion between rheumatology and pulmonology. Ultimately, it's felt that these findings are nonspecific with the potential for these to be a drug reaction, early interstitial lung disease, or of another cause. Because of this uncertainty, the methotrexate is discontinued, and azathioprine has started in its place. Over the coming months, the patient's articular disease remains very well controlled. She notices a little subjective improvement in her symptoms.

She's able to do a little more physically at work without trouble, and PFTs over one year later remain stable with her baseline assessment. In terms of imaging over time, unfortunately, she develops COVID nineteen pneumonia, and those, changes obscure any of the initial changes that were seen. So this case really highlights the point of what do we do when interstitial lung abnormalities are detected in a patient with rheumatoid arthritis? Now when we see findings of interstitial lung abnormalities such as reticulation, ground glass changes, one of the things that comes up right away is, is this RA interstitial lung disease? But other things on the differential include things like drug induced pneumonitis, particularly if a patient is on a medicine that we think of this frequently such as methotrexate.

And if that's the case, then the question comes up, what do you do with their current medications? Do you stop methotrexate in somebody who has incidentally detected interstitial lung abnormalities? Some important things to consider in this situation is that not all interstitial lung abnormalities or restrictive PFT findings are evidence of RA interstitial lung disease. So we just talked about that differential including other things such as drug induced pneumonitis, but also infections or post infectious reticulation can also appear similarly. People who have fluid overload perhaps related to heart failure may have some of these similar changes.

And then on PFTs we can have other causes of restrictive lung disease such as obesity, other chest wall diseases. Another important point is that when we incidentally detect these interstitial lung abnormalities or ILA, that doesn't necessarily mean that treatment changes are required. In some situations, after a detailed assessment of this patient, we might be able to keep them on their current therapies and just monitor them closely over time. But there are some things that the identification of an ILA does require in a patient with RA. And that is number one, we need to thoroughly assess this patient.

We need to ask about respiratory symptoms such as shortness of breath, cough, dyspnea of exertion. We need to obtain pulmonary function tests to understand their pulmonary physiology. These should be complete PFTs with spirometry, lung volume, and diffusion capacity. If available, it's always a great idea to have a multidisciplinary discussion with the pulmonologist and with the radiologist to discuss the potential etiologies of these findings. After doing these assessments, we don't wanna stop and never assess them again, but rather these assessments should be part of their ongoing care so we can understand the trajectory of their imaging findings, their pulmonary physiology, and their symptoms.

We need to do, we need to target the risk factors that can predispose these abnormalities to worsen or impact their survival. So things like if they're a cigarette smoker, we need to talk to them about smoking cessation. If they're exposed to other inhaled exposures, we need to mitigate those. We need to talk to them about optimizing physical activity levels. Now finally, when we detect an ILA, we also need to make sure that there weren't other findings on that CT scan we need to act on.

So other incidental findings of particular interest are lung nodules because patients with ILA are at higher risk of developing lung cancer. So those are the things that we absolutely need to do to really understand and monitor this disease over time. The things we may need to do require making treatment changes. But again, remember that not all ILAs are definitely RA ILD. Keep your differential open.

So thanks for joining me to discuss this case.

Hello, everyone. I'm Richard Conway from Dublin, Ireland, and this is, QD Clinics from RheumNow. And today's case is that of rapidly progressive rheumatoid arthritis interstitial lung disease or not. So this is the case of a 60 year old lady. She's been attending my rheumatology clinic for the last ten years.

She is an ex smoker. She has seropositive rheumatoid arthritis, strongly positive for rheumatoid factor and CCP antibody. So she is currently on treatment with methotrexate and adalimumab. And we have been lining her up in the clinic to switch over from the adalimumab to tocilizumab because she has not been controlled. So her disease activity has been high.

Her C. Dye at her most recent visit was 25. Her DAS 28 CRP is 5.4. So we have this plan to change her. We haven't changed her yet because she had some life things going on and wanted to delay treatment change.

So that plan has been there for about two months and hasn't happened yet. And I am surprised then to get a call from our Intensive Care Unit. And they tell me that this patient of mine has been admitted into them. And she come in initially through the emergency department. She's had a three to four week history of progressive dyspnea, which has culminated in her emergency department visit, a day or two on the ward, and she deteriorated further and has ended up in the intensive care unit, intubated and ventilated due to this pulmonary process.

She's had a chest x-ray and a CT scan done, and they show widespread pulmonary infiltrates with interstitial changes really throughout both lung fields. And they've called me and said to me that this lady has rapidly progressive rheumatoid arthritis interstitial lung disease. What do we do here? So that is the question. What do we do in a case like this?

I can tell from the way our intensivists are talking that they are keen for us to go ahead and start treating her autoimmune process. Maybe give her some methylbritinolone, maybe some rituximab, cyclophosphamide, something to address this perceived autoimmune process. I think it's always important to take a step back here. And that's what we did in this case. We said that, yes, absolutely, this would be oral ILD that has rapidly developed and progressed.

But that would be slightly unusual in somebody, yes, who's had high disease activity, but hasn't previously had interstitial lung disease and has had rheumatoid for quite a bit of time. And in some ways, it's not necessarily the type of patient we might be more worried about getting rapidly progressive lung disease in, which usually be older men who have relatively new onset disease. So we don't have a trajectory of the disease process over time, know, are still smoking. And that is not the case here. So a woman ex smoker, she's had disease for ten years or more.

So we look at her imaging and she really does have a lot of changes. Her lung fields on CT are there's no normal lung there. It's interstitial changes everywhere on the lung fields. And again, that could be seen with rapidly progressive rheumatoid lung disease, but would be a little bit unusual as a presentation. The other thing we note on this lady is that she does have quite a bit of herpes simplex around her mouth and nose.

Again, a sign that somebody is sick, potentially immunosuppressed, not unusual in an ICU patient, but worth noting. So our recommendation here was this before we do anything else and rush in with more immunosuppressive medicines, we should make sure that there's not something else going on. So we stop recurrent immunosuppression is one thing. And then do a bronchoscopy and some serum looking for potentially other infectious processes that could be at play here. So I know as rheumatologists, we are relatively wary of this and Pneumocystis jirovecii is always one that stands out to us and is in our minds.

But there are multiple other pathogens that could be at play here as well. And the other non infectious cause potentially is diffuse alveolar haemorrhage, unusual in rheumatoid arthritis, but can be seen in some of her other diseases. So this lady has her bronchoscopy. There's no blood staining of the sputum and bronchial or vage on that. Sent We it away to be cultured.

No signs of pneumocystis on it. But actually what comes back on her serum is that her CMV titers are absolutely through the roof. And so we felt that actually this lady probably had cytomegalovirus pneumonia. So viral opportunistic pneumonia from reactivation of the virus in the setting of immunosuppression. And then why would that happen?

That was a bit odd in the setting of somebody who's on a TNF inhibitor, methotrexate, yes, immunosuppressive, but something that we wouldn't consider overly so. And that brings us back to what was happening before she came in. She was due to switch, but hadn't switched due to life events. And she'd had a course of steroids from us, and then also had some further courses of steroids from her primary care, unknown to us in the interim. And these repeated use of oral glucocorticoids, it is a cumulative thing.

It does increase the risk over time. And if somebody is constantly getting pulses or tapers of these to control active disease, there is a risk there. So that is today's QD clinic. And our learning point from today is keep an open mind. Don't always presume it is the rheumatic disease that is causing the problem.

Tune into RheumNow for more from QD Clinics.

Hi, I'm Doctor. Janet Pope reporting at RheumNow. You can follow me on Twitter at JanetBurdope and welcome to QD clinic, a case that could be your patient. So this could be a patient with shortness of breath. So let's dive right in.

This is a 74 year old man. He has SEER positive RA and he has extra articular manifestations, nodules, subluxations with joint damage. Unfortunately, was a fifty pack year smoker, but he quit after having an MI when he was 70 years old. He's a retired high school teacher. He's on methotrexate, folic acid, and multiple cardiac meds.

However, he's also known to have chronic obstructive lung disease and he's on a puffer that's a combo of a long acting beta agonist and also an anti muscarinic inhaler. He has not needed steroids or antibiotics for his COPD. Now, he is an exerciser fortunately. So former smoker, he walks 10,000 steps a day because when he had his MI at age 70, he changed everything, stopped smoking, started walking regularly. He did undergo cardiac rehab and he knows to continue doing that.

He's never needed oxygen therapy. Now, just as an aside, when I have a patient who has obstructive lung disease and RA as a for instance, I do really look at vaccinations. So this fellow's vaccinations, is a retired school teacher and he's very forthright in keeping records. And he tells me and shows me that since he had his MI at age 70, every year he gets the annual over 65 year old flu shot. He did get his Prevnar twenty.

He had had Pneumovax before, but was able to get Prevnar twenty because he is immune suppressed and he doesn't wanna have a super infection. And he did get the RSV vaccine. Now, he has come to me though with the known COPD and he says he's more short of breath. He's not coming in because of that. It was a routine follow-up for his rheumatoid arthritis.

So when I auscultated, I heard bilateral basal crackles. And I looked back in my notes because I do make a habit of listening at the bases in every patient. And if you do it on every one, you may as well, you can find things and you can record it and then you know if there's a change. So that's a little clinical pearl. Listening to the bases is where the money's at for most of our patients for ILD.

So here's what I would do working this guy up. I would do a chest x-ray, not because I'm wondering about ILD initially, because I am wondering about that, but is there something like a bilateral pneumonia? I would consider if warranted his oxygen saturation at rest or with activity, I would order an HRCT scan. So he had an HRCT scan and it was a bit complicated and I did review it with the radiologist. So he had a mixed NSIP and UIP type changes.

So some honeycombing. He has emphysema as before, no significant bronchiectasis, and fortunately no pulmonary nodules. I also did PFTs and they were a mixed pattern. He had the known obstructive pattern and he also had a restrictive superimposed. So what are a couple pearls?

I don't want you to know how to read every PFT and I certainly don't know how to read PFTs. I just have a little bit of a sense about it because of running a large CTD practice. So obstructive, you're looking at FEV1 over FVC and the ratio should be reduced, less than 0.7. But if it's restrictive, there would be a reduced TLC, quite a markedly reduced DLCO often because it's parenchymal with the ILD. And you can also look at residual volume if you want, which would be elevated because of the obstructive lung disease.

So what are we gonna think about? We're going to look for other reasons for fibrotic or interstitial changes. So as a for instance, if this guy had amiodarone with a fib treatment, I would wonder about a drug effect. Of course, I am wondering if this is rheumatoid arthritis, but I would also look for eosinophilia, which could be a drug effect. So peripheral eosinophils on the differential.

I'd look for low grade infection, the atypicals, if he had sputum and fevers, if it was warranted. Things like Legionella are usually more acute, but post COVID he could have had COVID and then had chronic ILD changes. Mycoplasma pneumonia is usually acute, but it could be subacute. It can be bilateral and atypical mycobacteria. So there's a lot of things to think about.

He could also be more short of breath because of sleep apnea. So these things sort of come into my mind. And I think it's really important that we need a systematic approach and you can listen to some of the other modules in our CTD ILD unit and become more systematic in assessing your patients. So the bottom line is a mixed pattern is quite common. In rheumatoid arthritis with RAILD, about half of those patients, maybe a quarter to a half will also have emphysema because smoking is a risk for RA, RA ILD and for emphysema, but even in non smokers that can occur.

In systemic sclerosis about a fifth of patients with ILD from scleroderma will also have emphysema and a bit less so in the other CTDs. The final thing, if you really want to be keen about this is this can be called CPFE in CTD. So it's a combined pulmonary, both fibrotic or ILD and emphysema. But I don't think we have to worry about the names as much as really trying to diagnose and get the right treatment for the patient. Please follow us for the other reporting we'll be doing this month.

Thank you.