Inclusion Body Myositis

Overview

What is inclusion body myositis (IBM)?

Inclusion body myositis (IBM) is an inflammatory and degenerative muscle disease that causes painless weakening of muscle.

IBM gets worse slowly and is sometimes misdiagnosed as treatment-resistant polymyositis, another inflammatory muscle disease that causes muscle weakness. IBM also may be misdiagnosed as amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease).

Symptoms of the disease usually begin after the age of 50, although the disease can occur earlier. IBM occurs more frequently in men than in women and is the most common muscle disease in people aged 50 and older.

Symptoms and Causes

What causes inclusion body myositis (IBM)?

The cause of IBM is unknown. Because of the inflammation associated with IBM, some doctors think the disease is a form of autoimmune disorder. In this kind of disorder, the body’s immune system goes awry and attacks its own tissues – in this case, the muscles. Some experts have linked IBM to infection by a virus that has yet to be identified. Other researchers believe that the primary problem in IBM is an age-related inability of the muscle to deal with destructive chemicals.

What are the symptoms of inclusion body myositis (IBM)?

In IBM, the onset of muscle weakness usually is gradual, occurring over months or years. Falling and tripping usually are the first noticeable symptoms. For some people, IBM begins with weakness in the hands. People with IBM may have:

  • Difficulty with gripping, pinching, and buttoning.
  • Weakness of the wrist and finger muscles.
  • Atrophy (shrinking or wasting) of the muscles of the forearms.
  • Weakness and visible wasting of the quadriceps muscles (the large muscles on the front part of the thighs.)
  • Weakness of the lower leg muscles, below the knees.
  • Weakness of the esophageal muscles, which can cause dysphagia (difficulty swallowing) in about 30 to 40 percent of patients.
  • Weakness of other muscle groups as the disease progresses.

Diagnosis and Tests

How is inclusion body myositis (IBM) diagnosed?

Doctors use a muscle biopsy to diagnose IBM. After giving an anesthetic, a doctor takes a sample of tissue from one of the affected muscles to be looked at in a laboratory.

When viewed under the microscope, the muscle cells of persons with IBM contain vacuoles (rounded empty spaces). Within the vacuoles, there are usually abnormal clumps of several proteins including one called amyloid. The protein clumps, or inclusion bodies, give IBM its name. This is the hallmark of IBM.

Management and Treatment

How is inclusion body myositis (IBM) treated?

There is no effective course of treatment for IBM. The disease is not responsive to corticosteroids and immunosuppressive drugs – two drugs that often treat inflammatory or autoimmune conditions. Some evidence suggests that intravenous immunoglobulin may help slightly in a small number of cases, but the benefit does not last long.

Physical therapy may be helpful in maintaining mobility and helping to keep joints mobile. Other therapy, including treatments for the swallowing problems, is symptomatic and supportive.

Resources

For more information, please see the following:

Muscular Dystrophy Association - USA
National Office
161 N. Clark, Suite 3550
Chicago, IL 60601
www.mda.org
Toll free: 800.572.1717

Myositis Association
1940 Duke Street, Suite 200
Alexandria, VA 22314
Toll free: 800.821.7356
Email: tma@myositis.org
www.myositis.org

American Autoimmune Related Diseases Association, Inc.
22100 Gratiot Avenue
Eastpointe, MI 48021
Tel: 586.776.3900
Email: aarda@aarda.org
www.aarda.org

Last reviewed by a Cleveland Clinic medical professional on 04/02/2019.

References

  • Ropper AH, Samuels MA, Klein JP. Chapter 48. Diseases of Muscle. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor's Principles of Neurology, 10e. New York, NY: McGraw-Hill; 2014.
  • Dalakas MC. Chapter 388. Polymyositis, Dermatomyositis, and Inclusion Body Myositis. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.
  • World Gastroenterology Organisation Practice Guidelines. Dysphagia. Accessed 4/3/2019.
  • Genetic and Rare Diseases Information Center. Inclusion body myositis. Accessed 4/3/2019.

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy