Researchers Find a Path Forward for Patients With Polymyositis

Necroptosis, a type of regulated cell death, has been implicated in diseases such as inflammatory bowel disease, as well as neurodegenerative diseases.

According to a recent article in News Medical Life Sciences, a study found that using a treatment to inhibit necroptosis that negatively affects muscle fibers will reduce inflammation of muscles (myositis) and subsequent cell death.

The treatment involves a necroptosis inhibitor. It has the potential to treat a chronic skeletal muscle disorder that leads to a disability called polymyositis (PM).

Treatment for PM currently involves immunosuppressants to which some patients are not responsive. In fact, over half of treatment recipients do not see improvement. Therefore, muscle weakness continues to progress. Other negatives are infectious diseases that occur during treatment.

The need for improved therapy to suppress muscle inflammation, preserve muscle strength, and avoid infection is clear.

About the Study

Billions of cells will die every hour in the bone marrow and intestines of healthy individuals (apoptosis). The team of scientists conducting the study reasoned that by inhibiting muscle fiber cell death, they could suppress inflammation and muscle injury.

Using integrative analysis and muscle biopsy samples, the researchers confirmed that muscle fiber cell death is associated with necroptosis. When the muscle fibers are injured through necroptosis they release a protein that binds to DNA called high mobility group box 1 (HMGB1). The release leads to additional inflammation and muscle injury in patients with PM. To the researchers, it suggests that muscle cells are not passive but instead are aggressors and promote inflammation.

On a positive note, necroptosis and its molecular partners assist with postnatal development. Yet they also take part in cellular renewal. Necroptosis is now recognized as a pathway in various pathological conditions.

The researchers were encouraged when they observed that muscle fiber functions improved during treatment due to repression of necroptosis.

When looking at necroptosis involvement in various inflammatory diseases and its efficacy in inhibiting necroptosis in equivalent animal models, it has potential as a treatment for inflammatory myopathies. Such myopathies may appear in the heart, lung, skin, or gastrointestinal tract.

Given the promising effects in pre-clinical studies, clinical trials in PM are now under consideration.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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