Inclusion body myositis

Inclusion body myositis (IBM) is one of the most common disabling inflammatory myopathies in people over the age of 50. It falls under the group of conditions known as inflammatory myopathies, or myositis – “myo” meaning muscle and “itis” meaning inflammation.

IBM typically causes progressive muscle weakness, most often affecting the wrists, fingers and the muscles at the front of the thighs. It may also impact the muscles that lift the front of the foot, leading to foot drop.

In some cases, the muscles involved in swallowing are affected. This can lead to symptoms like choking while eating or drinking and, in more serious cases, frequent chest infections when food or fluids enter the lungs instead of the stomach.

There are two recognised subtypes of inclusion body myositis:

Inclusion Body Myositis

Types of IBM

There are two recognised subtypes of inclusion body myositis:

Sporadic Inclusion Body Myositis (sIBM):
The most common form, usually occurring in people over 50. sIBM is not inherited and typically begins with slowly progressing muscle weakness, often starting on one side. It commonly affects the quadriceps, finger flexors and ankle dorsiflexors. Swallowing difficulties may also occur. Muscle biopsies show inflammation, rimmed vacuoles and inclusion bodies. This type generally does not respond to immunosuppressive treatment.

Hereditary Inclusion Body Myopathy (hIBM):
Also known as GNE myopathy, this rarer form is caused by genetic mutations, most often in the GNE gene, and is inherited in an autosomal recessive pattern. Symptoms often begin earlier (in the 20s to 40s) and progress more slowly. Unlike sIBM, hIBM typically spares the quadriceps early on and does not show significant inflammation in muscle biopsy.

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