Announcer:
0:00
Welcome to the MedEvidence podcast. This episode is a rebroadcast from a live MedEvidence presentation.
Dr. Michael Koren:
0:07
For those of you that haven't been to these events before, we have sort of a formal teaching session for about 40 minutes and then we have about 20 minutes for question and answer. So we'll stick with that format. So here we go. The other thing we like to mention to the audience when we start is that, although you might think this is a free lunch, there is no such thing as a free lunch. So this is a lunch without a charge, but we're going to make you work for that lunch and we have a series of questions that we give to the audience. We want to understand your level of knowledge of certain things, your perspectives, and also to have some fun. So let's jump into some of these questions to start. So the first audience question is what is the primary function of the immune system? Multiple choice One hormone production to regulate growth and development. Two filter waste and excess fluids from the blood. Three defense from viruses, bacteria and infection. Four transmit nerve signals throughout the body. Or five keep rheumatologists employed.
Dr. Swati Shah:
1:24
The fifth one.
Dr. Michael Koren:
1:25
Who says one, okay. Who says one, who says two? Who says three? Smart audience. Who says four and who says five? We've got a few there. Well, I think when God developed the immune system, there weren't any rheumatologists around, but the answer, of course, is number three. So well done, audience. Okay, I'll let you jump in and educate us about understanding the immune system and immune diseases.
Dr. Swati Shah:
1:55
So that question segues this slide very beautifully. That immune system, as it seems like the name suggests, it's basically our immune system's defense against viruses or any invader or intruder. Autoimmune means your own immune system is acting up against you, so it is still fighting, it's still doing what it's supposed to do, but it's attacking the wrong guy. It's attacking your own cells. So, depending on which system of your body the immune system attacks, we have different names of autoimmune diseases and it can affect. That means it can affect every part of your body, every organ, different diseases. So we have a second question then.
Dr. Michael Koren:
2:45
All right. So that's a brief introduction to the immune system, and scientists continue to learn more about autoimmune diseases on a daily basis. True or false? Autoimmune diseases are a group of more than 100 chronic conditions. True. How do rheumatologists keep up with them? Actually, that's a joke from internal medicine training. You have to be really, really smart to be a rheumatologist because of so many diseases to keep up with. Or false. It's a government conspiracy.
Dr. Michael Koren:
3:18
So I'm going to go with true here, but how do?
Dr. Michael Koren:
3:22
you keep up with all these diseases
Dr. Swati Shah:
3:25
It's interesting. That's what led me into rheumatology, because it's very intriguing system and ultimately you could say there is one auto immune disease and you're just giving them hundred names. But it's important to recognize them separately because they do separate things. They're treated separately and separate different outcomes are possible. But it's very interesting. That's what I love about it.
Dr. Michael Koren:
3:51
Yeah, so I don't know how you keep up with them. We have maybe 10 things we treat in cardiology and that's challenging enough, but to treat 100 things is mind-blowing to me. Okay, next audience question On average, how long does it take to receive a diagnosis for an autoimmune disease? So people are laughing, so I think some of them may have gone through this process before. Okay, answer one, two and a half years and two physicians. Two, three years and three physicians. Three, four and a half years and two physicians. Two, three years and three physicians. Three, four and a half years and four physicians. Five years and three physicians 101 years and counting. So we'll just give you the answer.
Dr. Swati Shah:
4:42
And this is mind-blowing to me. So yeah, because of their heterogeneous diseases like let's take lupus, for example it can be we usually say there are 40 types of lupus and so it can be presenting itself so many different ways. Especially if you're not experienced with those diseases, it's very hard sometimes to diagnose.
Dr. Swati Shah:
5:07
Let's do our best
Dr. Michael Koren:
5:08
and it's rare that people would come directly to a rheumatologist that would have that knowledge base. So maybe several other physicians or they may have presented to an ophthalmologist first or even a cardiologist first, and we do our thing because that's what we see, and then we rule out our thing and then we pass it to the next person.
Dr. Swati Shah:
5:28
Correct.
Dr. Michael Koren:
5:30
Okay, so let's start with lupus, which I guess is probably the most common thing that you're treating.
Dr. Swati Shah:
5:35
So lupus is also called systemic lupus erythematosus or SLE. It's the quite autoimmune disease and it can affect an entire body. The most common form of lupus is SLE. The systemic nature of it, which means it affects the whole entire system, it can affect every organ, does not have to affect every organ all the time, but it can.
Dr. Swati Shah:
6:05
And what happens in lupus or any, for that matter, any autoimmune disease, but mainly in lupus is you require, or you usually have, a genetic predisposition. That means your genes either changed in your lifetime or you were inherited with those predisposing genes, and then they get turned on. So it's a two-hit factor. Well, you have a susceptibility and another, some trigger turns it on. Those triggers can be viruses and bacterias. But when it's turned on, it then in turn causes the immune cells, the T cells, the B cells, the all other immune cells, i to go into hyperdrive and they produce lots and lots of antibodies. Those antibodies are called autoantibodies because they recognize yourself as a foreign and then they start to affect that organ. Let's say, if it starts to affect the skin, then you would have skin rashes and that's the butterfly, commonly known butterfly rash on the face we see with lupus.
Dr. Michael Koren:
7:09
Are you usually able to identify the triggers, or often not?
Dr. Swati Shah:
7:13
Most of the time. Yes, we can't prove them, but we can suspect, because some viral infections happen and then within a few months, so it's really coincides with that trigger. So within few months you start to develop either joint swelling or skin rash. So you could have an idea, but there is no confirmatory test for that yet.
Dr. Michael Koren:
7:36
And then the types of lupus.
Dr. Swati Shah:
7:38
So the types of lupus. I kind of alluded a little bit earlier that there are kind of 40 types of lupus but the most common one is the SLE, the systemic lupus. The other one is cutaneous lupus and that only limits itself to the skin and it can still be devastating. It can still be affecting skin significantly but it mainly limits itself to the skin.
Dr. Swati Shah:
8:01
Drug-induced lupus is very important to recognize because it is caused by some other medicine, not necessarily just the medicine was given to you wrongly, the medicine was prescribed rightly, right dose and everything, and still it could lead to lupus. So medication, certain medications which cardiologists use as well, but many other medications endocrinologists use some antibiotics. They can also in some individuals, not in all, can trigger lupus and it's very important to recognize because when you discontinue that medicine the symptoms many times resolve. Neonatal lupus is very unfortunate, these little tiny babies, when they have lupus symptoms. But that's basically because the mother's autoantibodies transferred into the baby from placenta. So for first six months or so they can have symptoms similar to lupus. But that can they resolve spontaneously, thankfully.
Dr. Michael Koren:
8:57
Yeah, so in cardiology we used to use a medication called procainamide. Quite a bit back in the day when I was a cardiology fellow. It was a go-to medication for cardiac arrhythmias, including life-threatening arrhythmias, but we don't use it anymore because of these complications.
Dr. Swati Shah:
9:15
Hydralazine is another one
Dr. Michael Koren:
9:16
yeah yeah, not as common as procainamide in terms of the incidence of developing lupus, but, yes, one that we use for blood pressure and for congestive heart failure. What other drugs are you seeing that bring referrals to you?
Dr. Swati Shah:
9:28
Minocycline, the antibiotic used for skin conditions. Doxycycline rarely. And the one very frequently used for thyroid disease, the thiopurine and hyperthyroid medications.
Dr. Michael Koren:
9:44
Ah, gotcha. Okay, all right, so let's talk a little bit about common signs and symptoms.
Dr. Swati Shah:
9:51
So symptoms differ from person to person. So you may just want to look at your symptoms and discuss with your primary care or any provider, rather than trying to figure out if that's the common symptom any provider. But usually we see this rash Malar rash we call it which involves the cheek and the bridge of your nose and it spares the nasolabial fold. You can also have fever, fatigue, joint swelling, joint pain, muscle ache, chest pain, eye symptoms. You can have headache, because it's a vascular disease, and memory problem. When it affects CNS system, central nervous system, it can also make you prone to have clots, blood clots. It can involve mucous membrane and cause lots of mucosal sores and hair loss. So these are few to mention. But it can involve lots of other organs. So always check rather than and Raynaud's is another very common symptom, where the fingertips go dead white when exposed to even a little bit of cold.
Dr. Swati Shah:
11:00
So lupus causes and risk factors. So lupus does have female predominance, so 90% of patients are female. At the same time, if you're a male and you have lupus, it tends to be more aggressive, so you want to be very careful about that too. Usual age of presentation and development of lupus is 15 to 44, but we've seen it in very young kids and also into 70s as well. It is also genetically more seen in certain susceptible individuals. That patient may have family members with other autoimmune disease or lupus. It is also seen more frequently in African Americans, Hispanic and Asian Americans. And certain environmental risk factors are the triggers, which we were talking about a little earlier, and those are chemicals like smoke, silica, some viruses and bacterias, UV rays ultraviolet rays or sun rays are also one of the triggers. So many many times patients develop lupus symptoms for the first time after that great vacation in Cancun, Hawaii and stuff like that. So sunscreen is very important. Uv rays- yeah.
Dr. Michael Koren:
12:26
So I added one thing to your slide based on an experience I had. I was giving a talk about coronary disease and sex being a risk factor for coronary disease, meaning that men were more likely to get it than women and then after my talk, somebody came up to me and said Dr. Koren, you mentioned that sex is a risk factor for coronary disease, but you didn't say if it was yes or no. So I put that in as a little joke to amend your slides. Okay, Medications used to treat lupus.
Dr. Swati Shah:
12:57
We have for many years probably maybe I'm talking about 10 years ago. We used to say lupus has very limited treatment options. In the last 10 years we have had new treatment strategies for lupus and new developments and this is an exciting field now. So we still use those antimalarials, the hydroxychloroquine or quinine medicines we have been using for decades and it still is very helpful. But steroids are used for acute symptoms and as a bridge therapy until the actual steroid-sparing agent works. We also use non-steroidal anti-inflammatories time to time and then, because it's an autoimmune disease that means your immune system is causing it inappropriately we suppress immune system to treat it. Immune suppressive medications can be pills or injections or infusions, and blood thinners because it can cause blood clots many times.
Dr. Michael Koren:
14:00
Yeah, and just to point out, hydroxychloroquine has been famous recently because people thought it was a treatment for COVID and those clinical trials were done and unfortunately hydroxychloroquine does not work for COVID. But did you see less lupus during COVID crisis because of the use of that?
Dr. Swati Shah:
14:17
I know, but we saw a shortage of hydroxychloroquine during COVID time. Our patients with lupus had such hard time getting that medicine.
Dr. Michael Koren:
14:25
Interesting, so interesting. Okay, how about scleroderma? Why don't you educate us on that?
Dr. Swati Shah:
14:30
This is another very interesting disease and rare. This is luckily a little bit not as common as lupus. As in this picture you can see, there are nice wrinkles on the hands. Sometimes those wrinkles can be nice, like in scleroderma. You would love those wrinkles because what happens in scleroderma is the part of the skin which is involved gets very tight. All the wrinkles are gone. But that doesn't feel comfortable, that doesn't feel nice. So in this picture the, the ends of the fingers are taut and they look non-wrinkled compared to the other hand. So it's tightening of the skin.
Dr. Swati Shah:
15:08
But it can also involve blood vessels and joints, organs, lungs, heart, and it has mainly two types. We can differentiate between limited scleroderma and diffuse. Diffuse, as the name suggests, involves more part of the body and so usual symptoms of scleroderma. Some of them are similar as lupus, which is Raynaud's, the finger turning colors, but also arthritis, thickened skin. Skin gets thick, even on the face chest. It can involve the whole body. The joints may freeze up. You feel like when you woke up and you can't make a fist, you can't use your fingers. They're really taut for hours, not just for 5, 10 minutes. It can involve digestive system and cause gastroparesis and small bacterial, small bowel bacterial overgrowth and heart and lung as well.
Dr. Michael Koren:
16:07
Yeah, a devastating complication of scleroderma that, excuse me that I see on occasion, is pulmonary hypertension, which means very high blood pressure to the lungs, which can lead to intractable shortness of breath and incredible limitations of your day-to-day activities.
Dr. Swati Shah:
16:26
So what causes scleroderma? Your immune system inadvertently produces too much of collagen. It's not necessarily just too much, but it's also abnormal regulation of collagen, abnormal deposits of collagen in the skin that causes the skin to tighten up. Exact cause is not known, but again, it's some trigger, triggers your immune system with the genetic predisposition, and this combination of two hit factor one you were born with or changed in your genetics, and the trigger causes the initiation of the symptoms. Again, it is also more common in females. Many of our autoimmune diseases are common in females and that also raises a question that maybe hormones have something to do with it and there is some suggestion that as well. The age group where we see the scleroderma is usually 30 to 50. See the scleroderma is usually 30 to 50. African Americans can have earlier onset and also have more skin and lung involvement, including pulmonary hypertension.
Dr. Michael Koren:
17:32
So let's move on to rheumatoid arthritis, another common condition.
Dr. Swati Shah:
17:35
That's one of my favorite too and it's very common. In rheumatoid arthritis predominantly we see hands and feet, tiny, small joints, involvement and it can easily disfigure your fingers if left untreated and rheumatoid arthritis, you can think of it like they are your twin. I mean twin of your one cytokine called TNF, which is good as long as it's in limit. And when it is too much and causes too much of inflammation, then you have joint inflammation. It can also involve vessels too. So there is, it's normal part of our cytokines, but when it becomes too much or dysregulated, that's when you can get it.
Dr. Michael Koren:
18:21
And cytokines is a medical word which is a chemical, a protein chemical that tells cells what to do or encourages cells to do certain things. And we use the term cytokines for that Cyto meaning cell, kine meaning move Pretty cool.
Dr. Swati Shah:
18:37
Yeah, basically these proteins, the cytokines, cause everything to happen Every metabolism, every action of cells, good and bad. So if it's dysregulated then you get more inflammation. Otherwise it's perfectly in harmony and causes right amount of inflammation for right purpose. So rheumatoid arthritis usually you wake up with swollen, stiff joint. You can't hold your brush and try and brush your teeth it's very difficult. Opening doorknobs and opening bags are difficult. It causes significant fatigue because inflammation main symptom of inflammation is fatigue. Loss of appetite, low-grade fever can also happen.
Dr. Swati Shah:
19:25
Usually rheumatoid limits itself to small joints like hands and feet, but it can also involve spine, cervical spine and elbows, and hips and knees. It can also involve heart. Rheumatoid nodules can involve lungs. So it can have various symptoms depending on which is its main target. So if we talk about rheumatoid arthritis causes and risk factors, we're probably going into the same direction where it's your immune system which became dysregulated because of a trigger with a predisposition. But it has certain proven risk factors and one of them is smoking and the other one is viruses. Smoking or chemicals have shown to trigger lupus, cause those autoantibody formation and then it also leads to aggressive lupus. So if you continue smoking after diagnosis of rheumatoid arthritis, then it can also cause less response of medications, more damage of the joint and more aggression from rheumatoid arthritis. Women are more likely to get rheumatoid arthritis as well.
Dr. Michael Koren:
20:52
So I guess another good reason to stop smoking, if you do is to help your immune system out. All right, so let's get back to getting the audience back to work. What cells contribute to autoimmune diseases? Multiple choice Is it A cytokines we just talked about that B the rogue cell. C B lymphocytes, t lymphocytes and mast cells. D all of the above. And warning AI algorithm detects a trick question here. So the background is that Sharon, who you met in the beginning, said let's see if AI is better than the doctors at coming up with questions. So we actually asked AI a question and it was such a bad question I had to completely edit it. So the only thing that came up with it is the fact that it could be a trick question. So who thinks the answer to this question is A cytokines. Who thinks it's B the rogue cell. Who thinks it's C, b cells, t lymphocytes and mast cells. And who thinks all the above? See, yeah, I was right, it is a trick question.
Dr. Swati Shah:
22:05
We got a very smart audience.
Dr. Michael Koren:
22:08
And the reason it's a trick question is because the question asks what cells contribute to autoimmune diseases, and cytokines are a protein, not a cell. Go ahead if you want to comment on that.
Dr. Swati Shah:
22:22
No, this is a trick question. The audience is really smart, though, because majority did say answer C.
Dr. Michael Koren:
22:29
All right, so living with autoimmune diseases.
Dr. Swati Shah:
22:32
Living with autoimmune diseases is truly a challenge, but it can easily be navigated. You just need the right help and ask for help. So following your treatment plan absolutely necessary. Even if you think you have a healthy lifestyle, you can change your diet and do all of that, but still, if the treatment is necessary, follow the plans. Educate yourself. The more you know, the better you'll feel. The unknown irritates us, generates fear a lot. So the more you know, the better it is and you can ask more questions. You can be on right treatment and you can also choose the right treatment for you, because we all have different goals, different activity level, different plans. So different treatment for different individual is the right treatment. Sleep is very important. Stress is very important. Relieving stress is extremely important.
Dr. Michael Koren:
23:29
So both of us are very involved in clinical research and obviously we've certainly made tremendous progress for autoimmune diseases recently, but we all agree that there's a lot more that can be done. So maybe jump into some of the things that we're doing here locally and nationally and why we're excited about them.
Dr. Swati Shah:
23:46
So I have been with ENCORE Research Group for the last five years or so and I've been very happy because research is truly what helps patients, because we find out new therapies, new side effects, we find out how it works and, like we have right now a lupus study underway, rheumatoid arthritis study, scleroderma is under study. What we are doing is taking these diseases and trying to see what treatment works better. So if you have any disease or any side effects or any symptoms, you could try and figure out if there is a study going on and you could be part of it where you can help not just help yourself, but help others as well in this process.
Dr. Michael Koren:
24:33
Yeah, yeah. The other thing that I find interesting is there's sort of a cross-fertilization amongst the different specialties. So, for example, jak inhibitors are being used in inflammatory bowel disease and we were doing those studies, you know, five, 10 years ago, and now we know that they're safe and they work for one type of inflammation. The question is is does it work for another type of autoimmune disease or inflammation? So are you seeing a lot of that?
Dr. Swati Shah:
24:59
yeah, jak was very . We've been using in rheumatology field as well for rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and now we think it also works in lupus. There has been some studies which showed it did suggest good effect. Now we're just confirming with this study and they're pills the benefit of the JAK inhibitors. They're very simple to take, it's one tablet a day. Versus those infusions and injections, it's a very easy administration and you tolerate it really well as well.
Dr. Michael Koren:
25:32
Yeah, Now I think, and correct me if I'm wrong but I think we're not quite as advanced in treating scleroderma compared to lupus. Would that be accurate?
Dr. Swati Shah:
25:42
Very true, because scleroderma is a little bit of undiscovered pathophysiology, so exactly what happens in scleroderma is not very well known. That's why we're still suppressing immune system. But targeted therapies to that particular cytokine is missing. But we have a very good possible agent which we're using for scleroderma now and it is also easy to administer. So let's see if that works out. In preliminary data it has worked pretty well.
Dr. Michael Koren:
26:18
And some of these studies, as I understand, are enrolling now. So if people want to be screened or evaluated for those studies, let us know. The other thing we often learn is that people that get involved in research may find there's something else at the research facility that helps them. So, for example, people that have these autoimmune diseases may be at greater risk for having complications of influenza or other viruses, and we can help you with that as well. So it's not always the direct way of intervention that helps people, but sometimes an indirect way.
Dr. Swati Shah:
26:53
Explore. Yeah, I would say explore.
Dr. Michael Koren:
26:58
So how do you get involved? Give people a sense for how your patients have gotten involved and what their reaction has been.
Dr. Swati Shah:
27:06
So you can fill out forms, go onto the website or ENCORE Research Group and and just inquire, and you can also say what is suffering you, what is your interest, and our amazing staff can find you the right study or find you the study you're looking for. There is a free evaluation or screening visit so that you can be screened and that gives you an idea about what it involves before you decide. And this research is no cost to you and it offers compensation too. But it's more than that. It is the community service. It's like you're helping others.
Dr. Michael Koren:
27:48
Yeah, and that's a tremendous part of it is not only do we help our local community, but we also share information with people all around the world. So your participation as a patient in a clinical trial here in Jacksonville, Florida, is making people smarter the doctors smarter in Europe, in Africa, in the Middle East, in Asia, all over the world. So it's an amazing system we have of helping each other learn how to treat people.
Dr. Swati Shah:
28:14
So when you become a part of the study, that study is probably being conducted in Africa and Asia as well at the same time. So the whole data combined gives us a really great idea about what's going on.
Dr. Michael Koren:
28:25
Yeah, and Sharon mentioned about letting us have your email information. Again, we won't abuse that, we're not going to pass it along. But if we know your email and we know the type of things you're interested in, we can bring information to you that will probably be valuable to you. There's never an obligation. There's never anybody pushing you to get involved in these things. But one of my favorite statistics to quote is when you ask general patient populations would they be interested in a clinical trial, about 40% say that they have a strong interest in clinical research. But if you ask somebody that's done a clinical trial before, would they do a second one? Surveys show that 97% to 99% of people would do a second trial.
Dr. Swati Shah:
29:10
That speaks a lot. Yeah, that says a lot.
Dr. Michael Koren:
29:13
How many products can you think of that? Before you actually experience it you're a little skeptical, but once you experience it you say, yep, sign me up for the next one.
Dr. Swati Shah:
29:21
Correct yeah.
Dr. Michael Koren:
29:24
So we have a fun fact here. Go ahead and share that, if you would.
Dr. Swati Shah:
29:28
So it's a Latin word. Lupus is a Latin word for wolf. In those days, when you had that malar rash and scarring erosions on the skin, it mimicked wolf bite. So that's why the initial physicians, the earlier physicians, 13th century physicians, roharius, basically said this is wolf bite and that's how the name came about lupus.
Dr. Michael Koren:
29:56
All right, so I think we cover a lot of ground. Thank you for that amazing introduction to autoimmune diseases and thank you, audience, for your participation.
Announcer:
30:04
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