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What is necrotizing myopathy?

Necrotizing myopathy or also referred to necrotizing autoimmune myopathy is a rare muscular disease that is believed to be caused by a malfunction of the body’s immune system. The chief symptom that patient’s present with is a severe weakness of the muscles of the body.

People may find it difficult to climb stairs, get up from a sitting position, are unable to lift anything moderately heavy, and complain of fatigue on even the slightest of physical exertions. Some other symptoms that are associated with necrotizing myopathy include a difficulty in breathing, loss of weight, and loss of appetite.

Why is necrotizing myopathy caused?

The exact mechanism by which this disease is triggered in the body is unknown. Current research suggests that the use of statin medication, presence of a malignancy in the body, or an underlying connective tissue disease could be the possible causes for the initiation of necrotizing myopathy.

The presence of autoantibodies, which make the immune system target the body’s own muscles, have been detected in cases of necrotizing myopathy.

How is necrotizing myopathy diagnosed?

Clinical features of muscle loss without the presence of inflammation should alert the doctor to a possibility of necrotizing myopathy. A muscle biopsy will show plenty of muscle necrosis without otherwise expected inflammatory infiltrate in the area. The levels of the enzyme creatine kinase are markedly elevated in this condition as well.

A combination of the clinical symptoms, the presence of antibodies in the blood, and muscle biopsy is required to come to a definitive diagnosis of necrotizing myopathy.

Necrotizing myopathy can be pretty difficult to diagnose because it is a rare condition and is not the first thought that comes to a doctor’s mind. This diagnosis is often arrived at after a couple of the more common conditions have been ruled out.

That being said, the early detection and diagnosis are critical to the successful treatment of the condition.

How is necrotizing myopathy treated?

Necrotizing myopathy is believed to occur secondary to an underlying cause and so its treatment is focused on the identification and treatment of the root cause. Current treatment methods include the use of high-dose corticosteroids which have an immunosuppressive effect. A combination of corticosteroids with immunoglobulin g is currently the first choice of treatment in necrotizing myopathy and is effective in controlling the disease pretty effectively.

Some other patients may also need to put on a steroid-sparing immunosprresant such as methotrexate.

In very severe cases, rituximab has also shown to be beneficial.

The effects of the treatment being successful or not are measured through the measuring of muscle strength and measuring the creatine kinase levels in the body on a regular basis. Medicines are tapered off slowly and under close monitoring, because the risk of relapse of this condition is pretty high.

Conclusion

Necrotizing myopathy is a rare condition which can be potentially life-threatening. It is, however, treatable and patients that are diagnosed early have a good chance of managing the condition without any trouble.

  

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