Fibrosing Mediastinitis

Podcast Transcript

Raed Dweik:

Hello and welcome to the Respiratory Inspirations podcast. I'm Raed Dweik, chairman of the Respiratory Institute at the Cleveland Clinic. This podcast series of short digestible episodes is intended for patients and families and covers topics related to respiratory health and disease. My colleagues and I will be interviewing experts about timely and timeless topics in the areas of pulmonary, critical illness, sleep, infectious disease, and related disciplines. We will share with you information that will help you take better care of yourself and your loved ones. I hope you enjoy today's episode.

Dan Culver:

Hello and welcome to this episode of Respiratory Inspirations. I'm Dan Culver the chair of the Department of Pulmonary Medicine and I'll be your guest host for today's episode. We're very fortunate to have two guests with us today. Dr. Francisco Almeida, the head of the Fibrosing Mediastinitis Program here at Cleveland Clinic and Dr. Atul Mehta, the section head of the General Pulmonary Medicine section. Today we'll be talking about an enigmatic pulmonary disorder fibrosing mediastinitis. Welcome.

Dr. Francisco Almeida:

Hello Dan. Great to be here talking to you today.

Atul Mehta:

Hello Dan, looking forward.

Dan Culver:

So fibrosing mediastinitis is a mouthful. Can you give us a brief definition about what this syndrome is?

Dr. Francisco Almeida:

Well Dan, fibrosing mediastinitis is an uncommon condition characterized by a significant buildup of a dense scarring as we call in medically fibrosis or fibrotic tissue. And this scarring builds up in the mediastinum. The mediastinum is the area in the chest between the two lungs and also you can have this scarring buildup in the hilar areas. The hilar are the areas that sit sort of between the lung and the mediastinum or the area between the lobes of the lung, you know, the left lung has two lobes, the right lung has three lobes. So in between those areas. So that's pretty much the definition of fibrosing mediastinitis.

Dan Culver:

So, I imagine as the scarring builds up, it causes some problems. What does it do in the mediastinum when it builds up?

Dr. Francisco Almeida:

Correct. Correct. Correct. Yeah. So as this scarring builds up and it keeps growing and growing, it can compress important structures within the chest. Those structures can be vessels, so it can be large vessels or small vessels. And even small vessels can be impactful for the patients. It can narrow or occlude airways. So that can take away some of the air going in and out of the lungs. It can even compress the esophagus where the food goes into the stomach. So sometimes patients can have, trouble swallowing. So, between these compressions, the patients can cough up blood, they can have shortness of breath, they can have wheezing. Sometimes they can have tightness or chest pain. So that's pretty much what most patients end up feeling, depending on where the scarring develops and grows.

Dan Culver:

It sounds like a pretty challenging disease. And of course, the symptoms you describe can really be found in many different kind of pulmonary problems. If you're a patient listening to this, what should make you ask your physician about the possibility of fibrosing mediastinitis. What would lead the patient to be suspicious that this could be the problem going on?

Atul Mehta:

Yeah. The first thing is, as Dr. Almeida was referring to, that the main cause is a fungal infection. So, if you are from the area of Ohio, Mississippi, or Missouri River areas and your treatment for said pulmonary condition is not working properly. For example, you're told that you have asthma but the asthma medications are not helping you. Under those circumstances, and if you have had history of fungal infections or histoplasmosis, I think those patients should ask their physician a question, could this be the side effects or could this be the progression of my fungal infection of histo, could this be leading to fibrosis or scarring of my mediastinum producing shortness of breath.

If you have hemoptysis is a young patient where there is no risk for any other condition.

Dan Culver:

You mean coughing up blood.

Atul Mehta:

You may be coughing up blood, as a result of this along with shortness of breath. Yes, that also should raise the flag and say, could I have something like scarring in my mediastinum.

Dan Culver:

Of course, we see a lot of histoplasmoses here in Ohio and the areas you mentioned. I suppose that people are familiar with that. There are some other things that can cause this syndrome as well?

Atul Mehta:

Oh, absolutely. Other infections as tuberculosis, of course it is very rare in this country. But infections such as tuberculosis, we have large population of immigrants, so we should keep that thing in mind, can cause that. There are several other conditions which we refer to as collagen vascular disease such as Lupus, I'm sure you've heard the term, Lupus. Those collagen vascular conditions can also lead to scarring in the lung. A very rare cause, again in this country, once you keep it in mind, especially patients who have had HIV+ disease is syphilis can also cause fibrosing mediastinitis. And on occasion it is what we refer as unknown cause or idiopathetic fibrosing mediastinitis has been seen. If you have had prior radiation to your chest for Hodgkin's lymphoma or other lymphoma like conditions, that can also lead to scarring of the mediastinum.

Dan Culver:

So, lots of different things to keep in mind here.

Atul Mehta:

Absolutely. Yeah.

Dan Culver:

And as you mentioned, Francisco, there are many ways the patient can present. Can you talk a little bit about how the diagnosis is made and, you know, how the multidisciplinary team works together to make a diagnosis?

Dr. Francisco Almeida:

Yeah. So as Dr. Mehta mentioned, in patients who have certain symptoms that are not getting better, the classic example is probably asthma in which they are told they have asthma, and they're short of breath not getting better. So, the next step if not yet done is to get some type of imaging. And initially doctors may obtain a chest x-ray but a CAT scan of the chest is the test that most of the times will make a diagnosis. So, most patients don't even need a biopsy. The CAT scan findings in conjunction with where the patient comes from, the potential history of histoplasmosis. There are certain findings on the CAT scan that are classic of histoplasmosis infection, mostly prior infection. It's important to say here to the patients that the vast majority of fibrosing mediastinitis there's no active infection. The infection happened in the past and this is a reaction to that infection that keeps on going. So that's the main way of making a diagnosis.

However sometimes that CAT scan alone is not sufficient because there are other conditions that could mimic those findings. One of those conditions is like a cancer of the lymph nodes called lymphoma. So in these cases, we need help of our radiology colleagues to discuss with them. Sometimes we talk to our infectious diseases colleagues to say, "Hey, do you think there's an infection ongoing right now?" And sometimes we need the help of our other colleagues within the field. So sometimes we need to do a procedure called bronchoscopy with an endobronchial ultrasound to sample that lesion. When that's not possible, we may need to ask a surgeon to do a surgical biopsy or an interventional radiologist to do a biopsy through the chest. So, there are many ways that we work as a team to try to figure out the best way to confirm the diagnosis and to make sure we're not missing an alternative possibility.

Dan Culver:

So, you were a bronchoscopist. You mentioned bronchoscopy. Is that always a good idea? What are the kinds of things you do with a bronchoscope in patients with fibrosing mediastinitis and, you know, what do people need to know about before doing something like that?

Dr. Francisco Almeida:

Yeah. That's a great question, Dan. You know, even though I'm a bronchoscopist, I probably do that better than I ride a bike. Granted I'm not a great bike rider.

Atul Mehta:

That's not saying very much for sure.

Dr. Francisco Almeida:

Yeah. So that doesn't say a whole lot.

Atul Mehta:

I think I've seen him doing bronchoscopy.

Dr. Francisco Almeida:

But I'm pretty good I think at doing bronchoscopies.

Dan Culver:

I think so.

Dr. Francisco Almeida:

But this is a situation that I don't take the decision lightly of doing a bronchoscopy, actually for any patient. So, I sometimes, Dr. Mehta likes to say, you know, the best bronchoscopist is the one that knows when not to do it. And this is the type of patient where you have to be very careful. So doing this procedure by people who aren’t extremely experienced in doing bronchoscopy, it can be dangerous because these patients have a buildup of vasculature in the airways because of the blockage of the vessels. And that buildup can lead to bleeding on simple just little needles, just touching the area. They can have significant bleeding. So, if you don't have somebody, a bronchoscopist who works with an experienced anesthesiology team to deal with those bleedings, it can be life threatening.

Dan Culver:

That really can be quite dramatic when you look at it.

Dr. Francisco Almeida:

Correct.

Dan Culver:

Just visually, what does it look like to see an airway like that?

Dr. Francisco Almeida:

Oh, sometimes you go in and it- you know, the airway generally looks a little pinkish or, you know, a light reddish color. But sometimes you see these vessels that you feel like oh my goodness, what's going on in here. And they're so pronounced and so red that sometimes you are afraid of even looking at it because you are afraid that they're gonna bleed when that happens, let alone by touching it and biopsying it. So, it's very important for patients to, before they are recommended to undergo a bronchoscopy, to discuss with their doctor, you know, is this the best approach, you know, is there the support available in case I have an important bleeding.

Because sometimes when they bleed on occasion, the bronchoscopist, as good as they might be, they cannot control that right away. And having that multidisciplinary team, an interventional radiologist just down the hall to come, hey, we may need to block this vessel to stop that bleeding. So sometimes that's necessary. It's very rare that we get to that point here because of our experience in doing those procedures. But on occasion that becomes necessary.

Dan Culver:

So, you're really highlighting that experience is important. But the other thing that I think you mentioned and really, it's a part of the center is having multiple team members around the table and thinking about this from various different angles. Atul, tell me a little bit about who should be part of a team taking care of fibrosing mediastinitis patients? And how does the team work together to do that?

Atul Mehta:

I think this is a very good question for the community. The first member of the fibrosing mediastinitis team should be a pulmonologist, a lung doctor. That's number one. And he should be flanked by a new subspecialty of pulmonary medicine that is interventional pulmonology. Between two of them, they can suspect and confirm a diagnosis of fibrosing mediastinitis. And once this diagnosis is made, then other subspecialties need to get involved. Infectious disease specialty to make sure there is no active infection, or there are no other infections which we may not have thought about besides the fungal infection.

Then comes the role of interventional radiologist. These are radiologist beyond just diagnostic radiologist who can work through the vessels or the airways to help you open up obstructed passages or the blood vessels. This team should also include a thoracic surgeon just in case a thoracic surgery is required or some sort of a section or removal of the broncholith that is a stone inside the mediastinum or calcified lymph node within the mediastinum or the airways is required. So, you have to have a team of all these individuals helping us taking care of patients with fibrosing mediastinitis.

Dan Culver:

So, one of the things we've seen over the years is that various people do different interventions based on what their skillset is. We've seen people put little tubes inside the blood vessels called stents, we've seen stents inside of the breathing tubes. We've seen other kinds of medical therapies used. I wonder, Francisco if you can talk a little bit about what range of therapies, therapeutic options do you see as the most helpful? And especially the newer things that are coming out now.

Dr. Francisco Almeida:

Yeah. So, these therapies to open blockages are for the most part temporary. You know, for example a stent in the airway to open the airway. Stent in the airways, they can lead to complications, plugging of the airways by mucus. Sometimes there's some reaction of the airway, patients can have cough, other complications. So that's a temporary solution while there's a more permanent solution. Stents, you know, these devices to keep the airways op- the vasculature open or even the esophagus when needed can sometimes be permanent. But our goal should be to not need to use them. Of course, sometimes those are necessary no matter what, but our goal should be there a way that we can stop, you know, put a hold on that ongoing inflammation and potentially decrease that inflammation and decrease the size of that scarring tissue. So, the blockage is relieved, so it goes away.

So, in the past few years, people have been using a drug called rituximab, which is a drug that reacts or tries to prevent or decrease or stop that inflammation. And in some patients that drug can literally decrease that scarring which may avoid these other procedures. So that's our ultimate goal. Not everyone is a candidate for that therapy. That depends on certain aspects, on certain tests that we do. There's a test called PET scan, that we try to do to see how much inflammation there is in the scarring tissue, and which we can consider that drug as a possible treatment.

Dan Culver:

Yeah. I was going to ask you, why shouldn't everybody just take some rituximab and see what it does.

Dr. Francisco Almeida:

Correct. That's a really good question. So, when that inflammation is not shown in the PET scan, there's very little data right now to show that those patients may respond. We need more studies to figure out what we can do for those patients and which that scan called PET scan, P-E-T, doesn't show the inflammation. But when they show the inflammation, it seems like at least two-thirds of the patients have a good chance of responding and that inflammation decreasing or going away, and the scarring getting smaller and relieving that pressure to those compressions.

Dan Culver:

And I suppose rituximab is not without some risks.

Dr. Francisco Almeida:

Correct. It's not a chemotherapy drug, even though it can be used to treat certain types of tumors such as lymphoma for instance. But it's a drug also used for certain autoimmune disorders such as rheumatoid arthritis, for example. And it has some potential side effects. You know, there are many potential side effects, but most patients do well with it. But it's important to discuss with the patient the risks when that is considered, the pros and cons. But for the most part patients tolerate that well, but yes. Like anything we do in medicine, there are potential risks.

Dan Culver:

Atul, if somebody wants to come to the Fibrosing Mediastinitis Center, what can they expect? What will the process be like and what will their experience be like?

Atul Mehta:

We receive patients in two different ways. The patients will cover a variety of symptoms and we diagnose fibrosing mediastinitis on those patients. Those patients would expect to see an infectious disease specialist, would require a CT scan of the chest, would require probably a CT scan of the chest with the contrast material that to like at the vasculature or the anatomy of the mediastinum to see all the vessels are open. That is what is required. And if we find any obstruction, patient may require to see an interventional radiologist as well, virtually followed by a procedure. And then these patients will be followed for a long time in our outpatient department. Meanwhile, we may or may not treat this patient with oral medication such as antifungal medications or cortisone in that fashion.

I just want to point out that not everybody is a candidate for rituximab as Dr. Francisco mentioned. And want to point out that rituximab is an intravenous medication. It is given by infusion. It is an expensive medication. And it is not just writing a prescription that you get the medication; your insurance company will have to give us authorization before we can use that medication. That treatment is over several weeks, you know, we repeat the treatment again in six weeks, and then if required every four to six months that medication will be given to you in that particular fashion.

If you come to us with a diagnosis of fibrosing mediastinitis, the process is much quicker and shorter because we already have the diagnosis and we will take it in that fashion. Patient will be seen either by Dr. Francisco Almeida or myself, in the outpatient department with our team. We have certified nurse who is also helping us coordinate looking after the patients with fibrosing mediastinitis, Hilary Peterson is helping us taking care of these patients as well. We are keeping tab of every patient we see in our department and we remain in contact with them to see how they're progressing. I hope I'm answering your question.

Dan Culver:

Thanks. I think really the key is assessing the patient as an individual and then getting the multidisciplinary specialists around the table to think about how to best approach the diagnosis and the therapy. And that is something that I know is hardwired into the program very much.

Atul Mehta:

And we try to do this thing in a single or, you know, two or three visits to the Cleveland Clinic. We try to coordinate their visits to the Cleveland Clinic in the shortest period of time, the less frequent visits to the Cleveland Clinic.

Dr. Francisco Almeida:

Yeah. And just to add patients can assess our website, the fibrosing mediastinitis website, where they can even upload some of their data that they have from their local physician, local hospital and our phone number is there, easy to reach out to us and we get patients in very quickly when they need to be seen.

Dan Culver:

One feeling about fibrosing mediastinitis that's a sentiment out there still I think and certainly it's been there in the past has been a bit of nihilism. That there's nothing to be done, that this is really something you have to live with. Can you talk just at the end here a little bit about the prognosis nowadays with fibrosing mediastinitis. What do you see and where do you see the future going?

Dr. Francisco Almeida:

That's a great question, Dan. Yes, we have seen a lot of pessimism about these patients but now I think there's some hope, especially with some patients being potential candidates for this treatment we discussed, infusion. So those patients can change significantly their quality of life if they are candidates and receive the drug, and if they respond. Having said that, most patients their lifespan is not changed by the disease. So, patients who have disease predominantly on one side or the other of the chest, they can have pretty much the same lifespan of anyone who is completely healthy. If they have predominantly- if their disease affects both sides, that may be impacted. But even those patients can have a normal lifespan.

Obviously, our goal is to make them feel better and we have many options between this drug that we discuss and procedures that done through interventional radiology or interventional cardiology to open up vessels that are occluded or sometimes if the esophagus is occluded to stretch and open up the esophagus. So, there are a lot of options for patients to feel better. Yet, for the most part there's no cure unfortunately, except the occasional patient that has an area that can be taken out by surgery. The surgeon can go in and take that out, but that's generally not the approach we take for most patients.

Dan Culver:

Thank you. Okay. Atul, give us one final word, what one take home message would you like to leave the audience with today?

Atul Mehta:

Yeah. In recent years there has been significant advancement in radiology, interventional radiology, bronchoscopy, interventional cardiology, that we can relieve the symptoms of patients with fibrosing mediastinitis and improve their welfare.

Dan Culver:

That's good for hope. Francisco, one final word.

Dr. Francisco Almeida:

Yeah. I echo that. I want to tell the patients that we're here to work with you and to be your advocates in the process of the work up of this condition. And we always thrive as a team to make you feel better of this condition that has been very challenging, and is very challenging for patients, their families, and doctors who care for them. But we're here for you and we'll be happy to take care of you, like you're a family member.

Dan Culver:

Thank you. I want to thank you all for joining us today for Respiratory Inspirations. Thank you to doctors Almeida and Mehta. Have a good day.

Dr. Francisco Almeida:

Thank you.

Atul Mehta:

Thank you.

Raed Dweik:

Thank you for listening to this episode of the Respiratory Inspirations podcast. For more stories and information for the Cleveland Clinic Respiratory Institute, you can follow me on Twitter @RaedDweikMD.