What is the treatment for inclusion body myositis?

Treatment for Inclusion Body Myositis

There is no effective treatment that can reverse or significantly slow the progression of inclusion body myositis (IBM), with physical therapy and supportive care being the mainstay of management. 1, 2

Current Treatment Approach

Physical Therapy and Supportive Care

• Physical therapy should be instituted early to prevent joint contractures and muscle atrophy
• Goals include regaining muscle strength and function through stretching and strengthening activities 3
• Early referral to a physiatrist is essential to determine appropriate assistive devices 3
• Supervised exercise programs monitored by a physiotherapist are recommended to improve muscle performance 1

Pharmacological Interventions

• Unlike other inflammatory myopathies, IBM shows poor response to conventional immunosuppressive therapies:
  • Corticosteroids are not effective for IBM, despite their efficacy in other inflammatory myopathies 1
  • Multiple clinical trials have failed to demonstrate significant benefits from various immunosuppressive agents 2

Evidence from Clinical Trials

• Interferon beta-1a showed no important difference compared to placebo in muscle strength (moderate-quality evidence) 2
• Methotrexate failed to arrest or slow disease progression (moderate-quality evidence) 2
• Intravenous immunoglobulin (IVIG) trials have shown inconsistent results:
  • An open-label study found no objective improvement in muscle testing or functional disability scores 4
  • Other trials were unable to provide conclusive evidence of benefit 2

Dysphagia Management

• Dysphagia is a prominent feature in IBM that can lead to poor outcomes
• Regular assessment and interventions to prevent aspiration pneumonia are recommended 1
• Consultation with speech therapy and consideration of modified diet textures may be necessary

Monitoring Disease Progression

• Regular assessment using validated tools:
  • Inclusion Body Myositis Functional Rating Scale (10-point disease-specific functional scale) 3
  • Assessment of muscle strength and functional capacity
  • Monitoring for dysphagia complications 1

Multidisciplinary Care

• A comprehensive team approach is recommended, including:
  • Neurologist or rheumatologist
  • Physical therapist
  • Occupational therapist
  • Speech therapist (for dysphagia)
  • Specialist nurse 1

Common Pitfalls in IBM Management

1. Misplaced reliance on immunosuppression: Unlike other inflammatory myopathies, IBM typically does not respond to immunosuppressive therapies. Continuing ineffective treatments exposes patients to unnecessary side effects 1, 2, 5.

2. Delayed referral for physical therapy: Early physical therapy is crucial to prevent contractures and maintain function. Delaying this intervention can lead to accelerated functional decline 3, 1.

3. Overlooking dysphagia: Swallowing difficulties are common in IBM and can lead to aspiration pneumonia if not properly managed 1.

4. Failure to provide assistive devices: As IBM progresses, patients will require assistive devices. Early assessment for these needs can improve quality of life and prevent falls 3.

5. Unrealistic expectations: Setting realistic expectations about the progressive nature of IBM is important for both patients and providers, as no current treatment has been proven to halt disease progression 2, 5.

Despite numerous clinical trials of various immunosuppressive and immunomodulatory agents, there remains no proven effective pharmacological treatment for IBM. The focus should be on supportive care, physical therapy, and management of complications to maintain quality of life for as long as possible.