Inclusion body myositis: Difference between revisions

From WikiMD's Wellness Encyclopedia

← Older editNewer edit →
CSV import
No edit summary
Line 1: Line 1:
{{Short description|A progressive muscle disorder}}
{{Short description|A progressive muscle disorder}}
{{Use dmy dates|date=October 2023}}
{{Infobox medical condition (new)
| name            = Inclusion body myositis
| synonyms        = sIBM
| image          =
| caption        =
| pronounce      =
| field          = [[Rheumatology]], [[Neurology]], [[Neuromuscular medicine]]
| symptoms        = Progressive [[muscle weakness]], muscle wasting
| complications  = Mobility issues, difficulty swallowing ([[dysphagia]])
| onset          = Typically after age 45
| duration        = Chronic, lifelong
| types          = Sporadic inclusion body myositis (sIBM), Hereditary IBM
| causes          = Unknown, possibly autoimmune and degenerative
| risks          = Advanced age, genetic predisposition
| diagnosis      = [[Muscle biopsy]], clinical assessment, electromyography (EMG)
| differential    = [[Deconditioning]], [[hereditary muscle diseases]], [[polymyositis]], [[dermatomyositis]]
| prevention      = None known
| treatment      = Supportive therapies (physical therapy, occupational therapy), symptomatic management
| medication      = Immunotherapy generally ineffective; symptomatic medications as needed
| prognosis      = Slowly progressive disability, typically non-fatal
| frequency      = 5-71 per 1,000,000
| deaths          = Rarely directly fatal; associated complications can increase risk
}}


'''Inclusion body myositis''' (IBM) is a progressive [[muscle disorder]] characterized by muscle inflammation, weakness, and atrophy. It is one of the most common types of [[inflammatory myopathy]] in adults over the age of 50. IBM is distinguished by the presence of [[inclusion bodies]] in the muscle fibers, which are abnormal clumps of proteins.
'''Inclusion body myositis''' ('''IBM'''), specifically '''sporadic inclusion body myositis''' ('''sIBM'''), is a chronic, slowly progressive inflammatory muscle disease characterized primarily by progressive [[muscle weakness]] and wasting. It predominantly affects older adults, typically beginning after the age of 45.


==Pathophysiology==
==Signs and Symptoms==
Inclusion body myositis is considered both an [[autoimmune disease]] and a degenerative muscle disorder. The exact cause of IBM is not fully understood, but it involves an abnormal immune response that attacks the muscle tissue. This leads to chronic inflammation and the formation of inclusion bodies within the muscle fibers. These inclusion bodies contain proteins such as [[beta-amyloid]], [[tau protein]], and [[ubiquitin]], which are also associated with [[neurodegenerative diseases]].
Symptoms generally develop slowly and include:
* Progressive weakness in the forearms, wrists, thighs, and muscles controlling finger flexion
* Difficulty swallowing ([[dysphagia]])
* Gradual muscle atrophy, especially in the quadriceps and forearm muscles


==Clinical Features==
==Causes==
IBM typically presents with progressive muscle weakness and atrophy. The disease often affects the [[quadriceps]] muscles, leading to difficulty with activities such as climbing stairs and rising from a seated position. The [[forearm]] muscles and [[finger flexors]] are also commonly involved, resulting in difficulties with gripping and fine motor tasks. Unlike other inflammatory myopathies, IBM often affects the muscles asymmetrically.
The exact [[cause]] of IBM remains unknown. The condition is thought to involve both autoimmune and degenerative processes, possibly influenced by genetic factors.
 
==Risk Factors==
Factors increasing the likelihood of developing IBM include:
* Advanced age, typically over 45
* Genetic susceptibility


==Diagnosis==
==Diagnosis==
The diagnosis of inclusion body myositis is based on a combination of clinical features, laboratory tests, and muscle biopsy findings. [[Creatine kinase]] levels may be mildly elevated. Electromyography (EMG) can show characteristic patterns of muscle involvement. A muscle biopsy is crucial for diagnosis, revealing the presence of inflammatory infiltrates and inclusion bodies within the muscle fibers.
Diagnosis typically involves:
* Clinical evaluation and detailed patient history
* [[Electromyography]] (EMG) to assess muscle activity
* [[Muscle biopsy]] revealing characteristic inclusions and inflammation
 
Differential diagnosis should consider:
* [[Deconditioning]]
* [[Hereditary muscle diseases]]
* [[Polymyositis]]
* [[Dermatomyositis]]


==Treatment==
==Treatment==
There is currently no cure for inclusion body myositis, and treatment options are limited. [[Corticosteroids]] and other immunosuppressive therapies are generally ineffective. Management focuses on supportive care, including physical therapy to maintain muscle strength and function. Assistive devices may be necessary as the disease progresses.
There is currently no curative treatment for IBM. Management primarily includes:
* Physical therapy and occupational therapy to maintain mobility and functionality
* Supportive interventions such as speech therapy for dysphagia
 
Immunotherapy typically has limited or no benefit in IBM patients.


==Prognosis==
==Prognosis==
The progression of IBM is generally slow, but it leads to significant disability over time. Most individuals with IBM will eventually require assistance with daily activities and mobility. The disease does not typically affect life expectancy, but it can significantly impact quality of life.
IBM progresses slowly, causing increasing disability but rarely affecting life expectancy directly. However, complications such as severe swallowing difficulties can contribute to health risks.
 
==Epidemiology==
IBM is a relatively rare condition, with prevalence estimates ranging from 5 to 71 cases per million people worldwide, making it one of the most common inflammatory muscle diseases in adults over 50.
 
==Prevention==
No known preventive measures exist for inclusion body myositis.


==Related pages==
==Related pages==
Line 24: Line 73:
* [[Muscle biopsy]]
* [[Muscle biopsy]]
* [[Neurodegenerative disease]]
* [[Neurodegenerative disease]]
 
{{Systemic connective tissue disorders}}
{{Myopathy}}
{{DEFAULTSORT:Inclusion Body Myositis}}
[[Category:Systemic connective tissue disorders]]
[[Category:Muscular disorders]]
[[Category:Muscular disorders]]
[[Category:Autoimmune diseases]]
[[Category:Inflammations]]
[[Category:Neuromuscular disorders]]

Revision as of 08:02, 27 March 2025

A progressive muscle disorder


Inclusion body myositis
[[File:|250px|alt=|]]
Synonyms sIBM
Pronounce
Field Rheumatology, Neurology, Neuromuscular medicine
Symptoms Progressive muscle weakness, muscle wasting
Complications Mobility issues, difficulty swallowing (dysphagia)
Onset Typically after age 45
Duration Chronic, lifelong
Types Sporadic inclusion body myositis (sIBM), Hereditary IBM
Causes Unknown, possibly autoimmune and degenerative
Risks Advanced age, genetic predisposition
Diagnosis Muscle biopsy, clinical assessment, electromyography (EMG)
Differential diagnosis Deconditioning, hereditary muscle diseases, polymyositis, dermatomyositis
Prevention None known
Treatment Supportive therapies (physical therapy, occupational therapy), symptomatic management
Medication Immunotherapy generally ineffective; symptomatic medications as needed
Prognosis Slowly progressive disability, typically non-fatal
Frequency 5-71 per 1,000,000
Deaths Rarely directly fatal; associated complications can increase risk


Inclusion body myositis (IBM), specifically sporadic inclusion body myositis (sIBM), is a chronic, slowly progressive inflammatory muscle disease characterized primarily by progressive muscle weakness and wasting. It predominantly affects older adults, typically beginning after the age of 45.

Signs and Symptoms

Symptoms generally develop slowly and include:

  • Progressive weakness in the forearms, wrists, thighs, and muscles controlling finger flexion
  • Difficulty swallowing (dysphagia)
  • Gradual muscle atrophy, especially in the quadriceps and forearm muscles

Causes

The exact cause of IBM remains unknown. The condition is thought to involve both autoimmune and degenerative processes, possibly influenced by genetic factors.

Risk Factors

Factors increasing the likelihood of developing IBM include:

  • Advanced age, typically over 45
  • Genetic susceptibility

Diagnosis

Diagnosis typically involves:

  • Clinical evaluation and detailed patient history
  • Electromyography (EMG) to assess muscle activity
  • Muscle biopsy revealing characteristic inclusions and inflammation

Differential diagnosis should consider:

Treatment

There is currently no curative treatment for IBM. Management primarily includes:

  • Physical therapy and occupational therapy to maintain mobility and functionality
  • Supportive interventions such as speech therapy for dysphagia

Immunotherapy typically has limited or no benefit in IBM patients.

Prognosis

IBM progresses slowly, causing increasing disability but rarely affecting life expectancy directly. However, complications such as severe swallowing difficulties can contribute to health risks.

Epidemiology

IBM is a relatively rare condition, with prevalence estimates ranging from 5 to 71 cases per million people worldwide, making it one of the most common inflammatory muscle diseases in adults over 50.

Prevention

No known preventive measures exist for inclusion body myositis.

Related pages

Systemic connective tissue disorders
General
Other hypersensitivity/autoimmune
Other


Symptoms and conditions relating to muscle
Pain
Inflammation
Destruction
Low ATP reservoir
Abnormal movement
Retrieved from "https://wikimd.com/index.php?title=Inclusion_body_myositis&oldid=6537717"