 encompasses a group of autoimmune diseases that has proven difficult to manage due its systemic nature. These conditions may involve muscles, skin, and even the lungs. Today, there still remain challenges in diagnosing and managing them.

Chester Oddis, MD, of the University of Pittsburgh Department of Medicine, is an expert in idiopathic inflammatory myopathies who presented at the Congress of Clinical Rheumatology East 2020 about clinical characteristics and treatment aspects of myositis. 

 had the opportunity to speak with him about the current challenges in treating these types of conditions, promising new therapies for disease management, and how the COVID-19 pandemic has affected clinical practice in this area of rheumatology.

 Can you give a brief overview of myositis?

 These are autoimmune diseases that not only affect muscle, but they are systemic in their nature. For example, it's very common to have muscle involvements and skin involvement in many patients — as well as lung involvement, which is what I talked about a lot extensively during my presentation.

So, these diseases can be challenging, because there is a lot of systemic manifestations. And it can actually be joint involvement and other problems, just like what occurs in the other systemic diseases in rheumatology, including lupus.

It's an autoimmune attack that causes a lot of different things, like muscle weakness, inflammation in the joints, and significant skin rash. Unfortunately, it is the lung disease that oftentimes is a factor that really can adversely affect these patients with morbidity and mortality.

What is the importance of accurately diagnosing and classifying these types of diseases?

 In terms of diagnosis, I think it's important to make sure you're dealing with a particular autoimmune disease because there are going to be some mimics. Now, it's easy, in a way, whenever there is a rash. That's because the rash indicates this is dermatomyositis, for example. But if a patient just has muscle weakness — they don't have a rash, they don't have lung involvement, they don't have joint involvement — then, you've got to make sure that you're dealing with an autoimmune disease as opposed to one of the mimics of myositis. They might be anything from muscular dystrophy, to a drug/medication reaction, to other things that can affect muscle. 

So, it's almost easier when they have a rash that's characteristic of dermatomyositis.

 What are the challenges that come up in treating and managing myositis?

 Well, first you've got to diagnose it. And I think the challenge in diagnosing it is that you have to have the right collaborators. It's very easy, as I said, if they have a bad rash, and they're weak, and they have particular laboratory tests, such as autoantibody markers that are characteristic of the disease.

Now, the challenge in managing it is really significant, because a lot of these patients don't respond to conventional medications like steroids and methotrexate. Therefore, they require other disease modifying, or immunosuppressive, medications — especially when they get the involvement of lung, skin, joint, and muscle.

So, the challenge is to make the diagnosis in some instances, but also to get the right mix of treatment. These patients can have refractory muscle, skin, and other manifestations.

Are there any new, emerging therapies in this domain that are potentially promising?

 What we have now that we didn't have before is a multitude of potential biologic medications. We all know that the standard medication site, like steroids and methotrexate, work. But now we have had available many things, like rituximab, which depletes the B cells.

Also, there are a lot more trials that are available that are actually targeting specific cytokines in myositis. We're doing a trial, for example, looking at abatacept, which attacks the T cell in an effort to see if that improves the lung involvement and the muscle involvement in patients with myositis.

Another biologic therapy that is coming out is tofacitinib, or Xeljanz. It's an oral medication that is approved for rheumatoid arthritis. We've reported a small series of patients where the skin remarkably, and nicely, improved.

So, we're going to try anything that's going to work for other autoimmune diseases in this particular disease. You can go through the list of biologics. And it seems to me that the ones that help psoriasis skin rash might also help the rash of dermatomyositis.

 To shift gears a bit, we've seen how the COVID-19 pandemic has largely affected certain practices in healthcare and within various specialties. What would you say is the biggest challenge that this pandemic posed for you — whether it's on the side on the healthcare provider or the patient?

 In rheumatology, I don't think it has been a dramatic effect. Everybody was thinking in the beginning, "Oh boy, these patients are all on immunosuppressive medication. We're going to have to stop them." But we didn't do that, and it was not the recommendation of the American College of Rheumatology to do that.

So, many of our patients remained on the medications that they were taking (biologics, steroids, methotrexate, etc.) even if they were significantly immunosuppressive. That wasn't an anticipated problem as one would expect.

What was a challenge for us with the pandemic is seeing the patient face-to-face. If you're treating a disease where the muscle strength is something you want to follow, then you can't check it. I can't check your muscle strength looking at you [through the computer]. I can try to do it and say, "Okay, are you weaker, are you stronger, what's the change since the last visit?" But the challenge has been the sensitivity of detecting change clinically.

But we can see rash. We can rely on the patient telling us if the rash is better. So, the challenge has been a bit more on management and on diagnosis if it's a new patient. We like to see them face-to-face.

So, I wouldn't say that treatment has been as much of a problem as the diagnosis and management part of it.

 Do you have anything you want to add related to what we talked about or perhaps didn't discuss?

 What I would emphasize, which I did during my talk, is that many patients don't even have the muscle part of myositis. It's really the challenge of the things like I talked about earlier that really makes it difficult and interesting for us.

You've obviously heard of lupus, and the designation is systemic lupus. Well, all of our diseases are as equally systemic as lupus. And that's the challenge that I emphasized. Because I even presented cases during my lecture that really didn't have significant muscle problems.

So, it's the management of the many different manifestations. And it's also the appropriate diagnosis in terms of looking at autoantibodies that I emphasized. Those autoantibodies, those markers in the blood, are not only good for diagnosis, but they are also good for prognosis. By knowing what antibodies a patient has, you can also predict what types of problems they may get into later on. It always helps you to categorize and follow that patient.

Articles

Congress of Clinical Rheumatology East

An expert discusses the difficulties in diagnosing and managing myositis, as well as how the COVID-19 pandemic has affected clinical care.

Chester Oddis, MD: Myositis, Diagnosis and Management Challenges

 to continue caring for patients virtually. But the technology comes with many challenges.

The primary concern is a lack of a physical exam, Daniel Albert, MD, of Dartmouth-Hitchcock, said in a recent interview with 

. Rheumatologists often rely on a physical examination to get a better look at the whole patient.

“It’s a very difficult transition for providers to care for patients without a physical exam,” he said.

There are workarounds, however, but they require ingenuity. For example, patients often show Albert how they walk, how they use their upper extremities for activities, and physical exam maneuvers can be done similarly to how they would be done in person, but they have to use a visual rather than sensory input. Sometimes rheumatologists can elicit tender points on an individual if they are asked to put pressure on certain areas.

Other concerns exist, such as patients who are simply not suited for a virtual appointment, whether it be because they have a visual impairment or less cognitive ability than is required to conduct such an interview virtually. Sometimes diseases are just too complex and there are too many comorbidities, or the patient has too severe of a disease.

Lastly, it’s important for patients to buy into the idea of virtual encounters. If patients are uncomfortable, you have to give them the opportunity to be seen in the clinic.

Videos

Three key issues with using the technology over in-person visits.

Daniel Albert, MD: Telemedicine Challenges for Rheumatologists During COVID-19

t, Jeffrey Curtis, MD, MS, MPH, of the University of Alabama at Birmingham, discussed how 

 must be more attuned to patient risk for 

CV risk can increase as a result of the rheumatic condition or certain therapies that may have an adverse effect on lipid profiles.

In this final part of our interview, Curtis elaborated a bit further on this shortcoming in rheumatological care and a potential direction for the specialty.

“Although rheumatologists are certain specialists, I think many of us feel rather compelled to get back a bit more to our internal medicine roots,” he said.

This would mean engaging more in CV risk management, such as prescribing appropriate therapies and more actively monitoring aspects of CV health.

Curtis attributed this limitation in care to knowledge and willingness gaps. He believed this assuming of greater responsibility would meet a pushback in the field. After all, rheumatologists are already spread thin with time pressures for treatment.

“It doesn’t have to be that rheumatologists are always the prescribers,” said Curtis. “I think in many offices it’s just not realistic. But we can think through how we can better comanage and how we can communicate more effectively.”

He concluded with a hope that rheumatologists can take on greater responsibility and opportunities to optimize patient outcomes.

Care of rheumatic patients must factor in cradiovascular risk and conditions.

Jeffrey Curtis, MD, MS, MPH: The Future of Rheumatological Care

This past spring, the American College of Rheumatology has published 

on best practices for managing reproductive health in patients with 

The goal of these recommended guidelines is to encourage open communication and inform shared decision-making between patients and their physicians on issues related to reproductive health, including pregnancies.

“This area of reproductive health in rheumatic diseases has become increasingly important as our patients are in better health because of our better therapies,” said Lisa Sammaritano, MD, of Weill Cornell Medicine and Hospital for Special Surgery, in an interview with 

As improved treatments and technologies have become available in this domain, it has become increasingly important for patients, rheumatologists, and primary care physicians to make decisions together about the best strategies for optimal care.

Sammaritano noted that rheumatologists should still actively engage in these conversations on reproductive health, even if they themselves feel unable to provide comprehensive information.

She emphasized the usefulness of the ACR recommendations in providing guidance for rheumatologists and patients in these discussions.

“There are really helpful guidelines and management suggestions that are easy to refer to if one needs that,” she said.

Sammaritano is the lead author on the ACR reproductive health recommendations.

The recently published American College of Rheumatology guidelines on reproductive health is a great resource for rheumatologists.

Lisa Sammaritano, MD: Final Thoughts, ACR Guidelines on Reproductive Health

Telemedicine has been a boutique enterprise for the last dozen years, but recently, due to the 

 pandemic, it has become front and center for many practitioners, including those focused on 

Daniel Albert, MD, of Dartmouth-Hitchcock, told 

 in a recent interview that many providers and patients have had to do a quick ramp-up for the tools necessary to conduct such care and be cared for in such a way.

“It seems like it’s been very appealing to both groups and hopefully it will continue in largely the current format rather than the one that was unfortunately highly, highly regulated with a lot of barriers before COVID,” Albert said.

For Albert, he has an electronic health record (EHR) with a variant for his institution which permits a great deal of flexibility in terms of communication between patients and providers. The EHR allows for written documents, videos, still photos, and verbal and written interchange.

The platform that allows Albert to have a seamless video interaction with patients is called Vidyo. There are other variants that may have improved video or audio. A newer variant could allow for more devices being connected to the same interphase, and on Albert’s end, other students and staff members can be a part of the call to facilitate the conduct of the counter.

Providers are being careful to stay away from non-HIPAA compliant platforms like FaceTime and Skype.

How rheumatologists use telemedicine during COVID-19.

Daniel Albert, MD: Telemedicine for Rheumatic Care

Unless there was an unusual emergency, interventional pain procedures were considered elective, Alan Kaye, MD, PhD, of LSU Health School of Medicine, told 

 in a recent interview. Most were canceled because of concerns about supplies and the doctors did not want patients to get exposed.

“Pain medicine has gone through radical changes with telemedicine becoming the norm,” Kaye said.

The pandemic has been a serious challenge, he added.

Research has been published touching on all of the issues related to treating pain during COVID-19.

While pain doctors and rheumatologists alike have needed to shift its focus and use telemedicine to treat patients, some rheumatologists may have already been leveraging the technology for consults with patients.

“Every doctor and every person in America and probably worldwide have been affected by COVID,” Kaye said. He added it should not be surprising the 

 2020 meeting was taking place virtually over laptops.

Hear more from Kaye in the video interview clip posted above.

Alan Kaye, MD, PhD: Treating Pain During COVID-19

Rheumatologists should be more actively engaged in the management of 

, Jeffrey Curtis, MD, MS, MPH, Professor of Medicine at the University of Alabama at Birmingham, talked about the current gaps in rheumatological care of patients with cardiovascular risk.

The big question in this domain of care is on whom, or which specialist, does cardiovascular risk management fall.

“There’s often this tension with rheumatologists who say, ‘I don’t really want to mess with managing blood pressure, or hyperlipidemia. I’ve got a limited amount of time to do smoking cessation and lifestyle counseling.”

Curtis considered this hesitation or pushback to be a current shortcoming in the field. In fact, he noted that rheumatologists may tend to see patients with high cardiovascular risk more frequently than other specialists or the primary care physician.

However, he acknowledged that there is an ever-so-slight shift in the medical landscape that is just beginning to take place, and which can go even further.

“I think there is a growing recognition that rheumatologists are probably on the frontlines, and they need to increasingly recognize that,” he said. “At a minimum, they can coordinate care with respect to cardiovascular risk factor stratification as well as mitigation in order to get these risk factors under control.”

An expert discusses the role of the rheumatologist in managing cardiovascular risk factors as well as current gaps in the field.

Jeffrey Curtis, MD, MS, MPH: The Responsibility of Cardiovascular Risk Management in Rheumatology

Due to inflammation in the joints and the body attacking joints and muscles, many 

It is important to keep abreast of the evolution of pain treatment and techniques like interventional pain management can be used.

In every community now, there are American Society of Interventional Pain-affiliated specialists, and rheumatologists, regardless of location, can go on the website and find 1 of the 5000 members to connect with, Alan Kaye, MD, PhD, of the LSU Health School of Medicine, said in a recent interview with 

There are various rheumatologic diseases and for many of them, there are a lot who have pain.

“We don’t want to be practicing in the 1970s, 1980s, and 1990s,” Kaye said.

While Kaye’s approach is interventional medicine, other therapies exist rather than opioids, and there are ways to connect to experts who can help guide rheumatologists to the best possible care for a patient experiencing pain.

“These people should be your partners for pain management of these very complicated rheumatologic patients,” Kaye concluded.

An expert explains resources for pain related to rheumatic conditions.

Alan Kaye, MD, PhD: The Intersection of Pain and Rheumatology

 pandemic has exposed new pathologies and conditions that were once unheard of or considered unlikely in the 

. Cases of multisystem Inflammatory Syndrome in Children (MIS-C) is a new condition that has recently emerged in pediatric 

“This is a new entity that seems to be a post-infectious process in COVID-19 that children seem to be at higher risk for than adults,” said Randall Cron, MD, PhD, Professor of Pediatrics and Medicine, University of Alabama at Birmingham, in an interview with 

In many patients, MIS-C presents features similar to Kawasacki disease, with coronary artery changes in a small subpopulation. Furthermore, shock and gastrointestinal illnesses can be a result of the syndrome if the child is sick enough. Features of a cytokine storm may also occur.

Cron explained that, in addition to treating the shock, it would be typical to manage the hyperinflammation. Because of the syndrome’s shared features with Kawasacki disease, the patient then might receive intravenous immunoglobulin to prevent other complications, such as coronary aneurysms.

“I don’t know who would have predicted MIS-C, or PMIS, or PIMS, or whatever you want to call it. I certainly didn’t predict it,” he concluded. “It’s a fascinating new entity, unfortunately, for pediatric rheumatologists and others.”

MIS-C is a condition recently recognized in children with COVID-19.


Randall Cron, MD, PhD: Multisystem Inflammatory Syndrome in Children

When considering pregnancy, patients with 

 must consult with their healthcare providers. Additionally, there must be an open line of communication between their rheumatologist and obstetrician-gynecologist (OB-GYN).

“Rheumatology patients have special concerns and risk with regard to pregnancy, and therefore it is very, very important that this be planned,” said Lisa Sammaritano, MD, of Weill Cornell Medicine and Hospital for Special Surgery in an interview with 

Systemic lupus and antiphospholipid antibodies syndrome historically have posed the greatest risk for both the mother’s health and the outcome of the pregnancy. Both disorders may coincide, thus increasing risk. In fact, about 1/3 of patients with lupus also present with antiphospholipid antibodies.

Sammaritano urged rheumatologists to inquire into their patients’ intentions, and to caution against inadvertent pregnancies. Furthermore, the ideal treatment and management scenario would be a level of synergy between both the rheumatologist and OB-GYN; both specialists must be attuned to patient conditions and pregnancy plans.

“We may think of this as the domain of the OB-GYN. Of course, they know everything about pregnancy and contraception. But we know everything about lupus and other related diseases,” she said. “And really, it takes both of those knowledge bases to come up with the right solution for any given patient.” 

Rheumatologists and OB-GYNs must communicate openly with each other and to patients with rheumatic diseases who are considering pregnancy.

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