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Managing dermatomyositis, a rare autoimmune disease causing muscle weakness

“I couldn’t speak, walk or sit.” Inside a rare autoimmune disease that attacks the muscles

Elisa Glass was diagnosed with dermatomyositis, which causes severe muscle weakness throughout the body. For Myositis Awareness Month, she shares her story.

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One week back in January 2016, Elisa Glass was feeling tired and off-kilter. That Thursday, she developed body aches and weakness that felt like painful charley horses in the back of her legs and went to her primary care physician. Her doctor initially suspected Lyme disease based on Glass’ symptoms and the fact that she lives in the Northeast (where the illness is prevalent). Armed with a diagnosis and a prescription, Glass hoped the muscle spasms would abate.

But by Sunday night, Glass couldn’t get out of bed. In addition to a heavy feeling in her legs, the now 62-year-old recalls, she had a rash on her face. “My skin was itching and on fire, and I looked like a snake shedding its skin.” The next morning, Glass couldn’t move her legs, and the rash on her face had spread to her hips, scalp and back of the neck. Her husband had to carry her to the local emergency room.

Headshot of Elisa Glass

Glass was diagnosed with myositis in 2016.

When she arrived, doctors quarantined Glass, thinking she had an infectious disease. After blood tests ruled out the initial Lyme disease diagnosis and doctors suspected mononucleosis, her case was sent to the rheumatology team. Two rheumatologists looked at Glass’ rash, assessed her symptoms and did thorough testing, which confirmed their suspicions: She had a disease called dermatomyositis.

Dermatomyositis (DM) is a rare autoimmune disease, part of a group of conditions called Idiopathic Inflammatory Myopathies (IIM). (The word “myositis” means “muscle inflammation.”) With IIMs, the body typically produces antibodies that attack the muscles and other connective tissue, including skin, causing inflammation and damage. The first sign of IIM is typically weak muscles; a patient might have difficulty standing, walking or picking things up, explains Terence Rooney, VP Disease Area Stronghold Leader, Rheumatology, Johnson & Johnson Innovative Medicine. In some extreme cases, muscles may weaken to the point of paralysis.

This chronic condition commonly affects the arm and leg muscles, but other parts of the body may be affected as well—like the muscles used to talk and even breathe. “It’s a multi-system connective tissue disease,” Rooney says.

There’s currently no way to prevent myositis, nor is there a way to predict it. There is also no cure for IIM, but there are treatments to help with symptoms.

Patients like Glass are why scientists at Johnson & Johnson are committed to gaining knowledge about this rare disease, as well as developing potential medications that may help treat it. For Myositis Awareness Month, here are five things to know about IIM, plus insights into Glass’ journey navigating the disease.


There are several different types of IIM

The three most common subgroups of IIM include DM, Immune-mediated necrotizing myopathy (IMNM) and Anti-synthetase syndrome (ASyS). These subgroups were established in 2017 by the European League Against Rheumatism and American College of Rheumatology, when it published new classification criteria for IIM that aims to distinguish it from other conditions.

DM can affect all ages and genders, but it is more common among women. Patients may experience muscle weakness, along with a rash on the eyelids, cheeks, nose, back, upper chest, elbows, knees and/or knuckles. IMNM involves muscle weakness with evidence of muscle cell death (necrosis). With ASyS, clinical symptoms include myositis, fever, Raynaud’s phenomenon, nonerosive arthritis, mechanic’s hands and Interstitial lung disease (ILD).

There is no straightforward, quick diagnosis for this disease, especially because there are sometimes multiple organs involved.
Cathye Shu
M.D., Ph.D., Director of Immunology Clinical Development, Johnson & Johnson Innovative Medicine

IIM tends to strike children younger than 15, as well as people between the ages of 45 and 54, with slightly more women getting diagnosed than men. In people under age 50, DM is more common.


Some patients with IIM may go undiagnosed for years

Diagnosing IIM is challenging because they are highly variable diseases. Patients can often be misdiagnosed or treated for other diseases before a proper diagnosis is made.

An estimated 2 to 8 people per million are diagnosed with IIM annually, sometimes after years of suffering without knowing the cause. “There is no straightforward, quick diagnosis for this disease, especially because there are sometimes multiple organs involved,” says Cathye Shu, M.D., Ph.D., Director of Immunology Clinical Development, Johnson & Johnson Innovative Medicine. “IIM can affect the skin, joints, lung, cardiac and gastrointestinal systems. In some cases, diagnosis can take up to 10 years.”

Creatine Kinase enzyme, found in brain, skeletal, and heart muscles

Close-up shot of creatine Kinase enzyme, found in brain, skeletal, and heart muscles.

Diagnosis can require a multi-pronged approach, often including a thorough exam and muscle strength and coordination tests. Patients may undergo blood tests to check for elevated levels of muscle enzymes such as creatine kinase (an intracellular enzyme found in the brain, and in skeletal and heart muscles), which can indicate muscle deterioration. Doctors may also take a muscle biopsy to test the tissue for inflammation and rule out other conditions, such as muscular dystrophy.


IIM may cause organ damage

For IIM patients, there is a very real risk of organ damage, including to the lungs, explains Rooney. “When disease impacts the muscles that help us breathe, it can be life-threatening.” It’s estimated that 5% of people with dermatomyositis die from complications of the disease within a year of diagnosis.

My body was shutting down; my family was coming to see me, to say goodbye.
Elisa Glass

Even after receiving treatment, Glass’ health deteriorated. At first, steroids helped reduce her pain and symptoms, but six weeks after diagnosis, she lost her voice and her ability to eat. “I couldn’t speak, walk or sit. My trach tube wasn’t staying in,” she recalls. “My body was shutting down and my family was coming to say goodbye.”

But according to Dr. Shu, relapses like Glass’ aren’t uncommon. “If patients don’t have early treatment, muscle inflammation can lead to permanent damage and loss of mass that necessitates assistive devices such as canes or wheelchairs,” she says. The sooner the IIM can be controlled, the lower the chances of complications.


A combination of medicine and exercise can help patients stay in remission

Historically, the most effective treatments for IIM have been high doses of immunosuppressants and corticosteroids to treat the inflammation, Rooney explains. But that’s not practical for long-term disease management, he explains, because corticosteroids have well-known severe side effects—including infection, osteoporosis and metabolic disturbance—when used for extended periods of time. There’s a desperate need for a more targeted approach.

“What we really need is a safe and well-tolerated medication that will shut down the attack—one that doesn’t just treat symptoms, but that treats the root cause of disease,” says Rooney.

Currently, Johnson & Johnson is developing a therapy that targets the specific antibodies that attack the muscles. “This targeted therapy is designed based on an understanding of how IIM develops,” Rooney adds, so the therapy can deliver a surgical strike at the part of the immune system triggering this response. The goal: to reduce the levels of pathogenic antibodies—also known as autoantibodies—associated with this disease.

“If we do that, we believe symptoms will improve,” says Dr. Shu, who is leading clinical trials. “Then patients could follow a chronic maintenance treatment, which may prevent relapse or disease flare-ups.”

Treating the inflammation is one part of the equation—it’s also important to exercise the muscles to keep them as strong and flexible as possible. Patients may also benefit from other sources of support. Once Glass was released from the hospital, she began to receive physical therapy and speech therapy at her home, and she saw a neuropsychologist and social worker regularly. She credits the combination of a physical and mental approach with helping her heal. “It’s helped me in my journey, to find myself and define my mission.”


IIM is a rare condition and doctors are trying to learn more about it
Things We Now Know About IIM-warrior-0424.png

Eight years after her first myositis flare-up, Glass is managing her symptoms and feels lucky for what her body can do.

The medical community has made strides in IIM research in recent years. But there are still questions to be answered. “We have yet to understand what kicks these diseases off in the first place,” says Rooney. With greater understanding could come even more customized care. “Having a better understanding of an individual patient’s unique ‘flavor’ of this disease-specific symptoms, for example—could help us further personalize therapy.”

Eight years after her first DM flare-up, Glass is managing her symptoms and feels lucky for the things her body can do. “I can’t run like I used to, but I’m walking, I’m talking and I’ve learned how to manage my illness,” she says. “I was the most severe case my medical team had ever seen, and they are shocked that I am still alive.”

Glass is now an advocate for IIM; she runs resource and support groups around the world to help people with chronic disorders manage their illnesses. “Everyone who has a rare disease thinks we are misunderstood or ignored because we are ‘rare.’ Who would notice us? But when you put all the ‘rare’ people together, you see there are many of us. And there’s power in numbers.“

Are you living with a rare disease?

See if you might be eligible to participate in a study that could potentially help lead to more effective treatment.

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