Inclusion body myositis
Updates to Article Attributes
Inclusion body myositis (IBM) is a type of inflammatory myopathy. It is often considered is the most common acquired myopathy in patients older than 50.
Epidemiology
It tends to present in older individuals 4 (often after the age of 50), although the disease may occasionally present earlier. There may be a greater occurrence in males (M:F up to 3:1) 6.
Associations
Recognised associations include:
-
diabetes mellitus:
~ 20~20% 6 - other autoimmune conditions:
~ 15~15% 6
Clinical presentation
The onset of symptoms is generally gradual (over months or years). Falling and tripping are often the first noticeable symptoms. In some cases individuals, the disorder begins with a painless weakness in the wrists and fingers that causes difficulty with pinching, buttoning, and gripping objects. There may be weakness of the wrist and finger muscles and atrophy (thinning or loss of muscle bulk) of the forearm muscles and quadriceps muscles in the legs. Difficulties with swallowing can occur in approximately half of cases.
Pathology
It is characterized by chronic, progressive muscle inflammation accompanied by muscle weakness.
Although it is classified as an inflammatory myopathy, inflammation is not considered a dominant component of the disease. The pathognomonic histologic feature of this condition is the presence of inclusion bodies in the nucleus and cytoplasm of affected muscle cells.
Location
It can affect both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles. It is usually bileteral although Muscle weakness may affect only one side of the body.
Usual muscles involved include:
- quadriceps 6
- iliopsoas
- tibialis anterior
- deltoids
- biceps brachii 6
- triceps muscles 6
- finger flexors
- ankle dorsiflexors
Radiographic features
MRI
May show evidence of muscle oedema although this is not a specific feature. Abscess formation is generally not seen 1.
More content required
Treatment and prognosis At the time of writing there is no definitive cure nor a standard course of treatment for IBM. The disease is generally unresponsive to corticosteroids and immunosuppressive drugs 4. There may be some evidence suggestive a slight ,short lasting benefit from intravenous immunoglobulin therapy. Management is essentially supportive (physical therapy to improve mobility etc). For MRI appearances consider:Differential diagnosis
-<p><strong>Inclusion body myositis (IBM)</strong> is a type of<a href="/articles/inflammatory-myopathy" title="inflammatory myopathy"> inflammatory myopathy</a>. It is often considered is the most common acquired myopathy in patients older than 50.</p><h4>Epidemiology</h4><p>It tends to present in older individuals <sup>4</sup> (often after the age of 50), although the disease may occasionally present earlier. There may be a greater occurrence in males (M:F up to 3:1) <sup>6</sup>. </p><h5>Associations</h5><p>Recognised associations include</p><ul>- +<p><strong>Inclusion body myositis (IBM)</strong> is a type of<a href="/articles/inflammatory-myopathy"> inflammatory myopathy</a>. It is often considered is the most common acquired myopathy in patients older than 50.</p><h4>Epidemiology</h4><p>It tends to present in older individuals <sup>4</sup> (often after the age of 50), although the disease may occasionally present earlier. There may be a greater occurrence in males (M:F up to 3:1) <sup>6</sup>.</p><h5>Associations</h5><p>Recognised associations include:</p><ul>
-<a href="/articles/diabetes-mellitus" title="diabetes mellitus ">diabetes mellitus </a>: ~ 20 % <sup>6</sup>- +<a href="/articles/diabetes-mellitus">diabetes mellitus</a>: ~20% <sup>6</sup>
-<li>other autoimmune conditions : ~ 15 %<sup> 6 </sup>- +<li>other autoimmune conditions: ~15%<sup> 6 </sup>
-</ul><h4>Clinical presentation</h4><p>The onset of symptoms is generally gradual (over months or years). Falling and tripping are often the first noticeable symptoms. In some cases individuals, the disorder begins with a painless weakness in the wrists and fingers that causes difficulty with pinching, buttoning, and gripping objects. There may be weakness of the wrist and finger muscles and atrophy (thinning or loss of muscle bulk) of the forearm muscles and quadriceps muscles in the legs. Difficulties with swallowing can occur in approximately half of cases. </p><h4>Pathology</h4><p>It is characterized by chronic, progressive muscle inflammation accompanied by muscle weakness. </p><p>Although it is classified as an inflammatory myopathy, inflammation is not considered a dominant component of the disease. The pathognomonic histologic feature of this condition is the presence of <a href="/articles/inclusion-bodies" title="inclusion bodies">inclusion bodies</a> in the nucleus and cytoplasm of affected muscle cells.</p><h5>Location</h5><p>It can affect both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles. It is usually bileteral although Muscle weakness may affect only one side of the body. </p><p>Usual muscles involved include</p><ul>- +</ul><h4>Clinical presentation</h4><p>The onset of symptoms is generally gradual (over months or years). Falling and tripping are often the first noticeable symptoms. In some cases individuals, the disorder begins with a painless weakness in the wrists and fingers that causes difficulty with pinching, buttoning, and gripping objects. There may be weakness of the wrist and finger muscles and atrophy (thinning or loss of muscle bulk) of the forearm muscles and quadriceps muscles in the legs. Difficulties with swallowing can occur in approximately half of cases.</p><h4>Pathology</h4><p>It is characterized by chronic, progressive muscle inflammation accompanied by muscle weakness.</p><p>Although it is classified as an inflammatory myopathy, inflammation is not considered a dominant component of the disease. The pathognomonic histologic feature of this condition is the presence of <a href="/articles/inclusion-bodies">inclusion bodies</a> in the nucleus and cytoplasm of affected muscle cells.</p><h5>Location</h5><p>It can affect both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles. It is usually bileteral although Muscle weakness may affect only one side of the body. </p><p>Usual muscles involved include:</p><ul>
-</ul><h4>Radiographic features</h4><h5>MRI</h5><p>May show evidence of muscle oedema although this is not a specific feature. Abscess formation is generally not seen <sup>1</sup>. </p><p><em>More content required</em></p><h2 id="Is_there_any_treatment">Treatment and prognosis</h2><div>-<p>At the time of writing there is no definitive cure nor a standard course of treatment for IBM. The disease is generally unresponsive to corticosteroids and immunosuppressive drugs <sup>4</sup>. There may be some evidence suggestive a slight ,short lasting benefit from intravenous immunoglobulin therapy. Management is essentially supportive (physical therapy to improve mobility etc). </p>-<h4>Differential diagnosis</h4>-<p>For MRI appearances consider</p>-<ul><li><a href="/articles/differential-diagnosis-of-skeletal-muscle-oedema-on-mri" title="Differential diagnosis of skeletal muscle oedema on MRI">differential diagnosis of skeletal muscle oedema on MRI</a></li></ul>-</div>- +</ul><h4>Radiographic features</h4><h5>MRI</h5><p>May show evidence of muscle oedema although this is not a specific feature. Abscess formation is generally not seen <sup>1</sup>.</p><p><em>More content required</em></p><p>Treatment and prognosis</p><p>At the time of writing there is no definitive cure nor a standard course of treatment for IBM. The disease is generally unresponsive to corticosteroids and immunosuppressive drugs <sup>4</sup>. There may be some evidence suggestive a slight ,short lasting benefit from intravenous immunoglobulin therapy. Management is essentially supportive (physical therapy to improve mobility etc).</p><h4>Differential diagnosis</h4><p>For MRI appearances consider:</p><ul><li><a href="/articles/differential-diagnosis-of-skeletal-muscle-oedema-on-mri">differential diagnosis of skeletal muscle oedema on MRI</a></li></ul>