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Inflammatory myopathies (IM)

Overview

IM are a collection of uncommon autoimmune disorders affecting the skeletal muscles. These conditions may also present with extramuscular symptoms such as skin rash, joint inflammation, interstitial lung disease (ILD), and heart-related complications.

  • Dermatomyositis (associated with characteristic skin rash- heliotrope rash, V sign, shawl sign, occasional dysphagia)
  • Overlap myositis (related to connective tissue disorders)
  • Antisynthetase syndrome (ASyS) (presents with multisystem involvement, including arthritis, ILD, and myocarditis)
  • Immune-mediated necrotizing myopathy (IMNM) (muscle predominant)
  • Sporadic inclusion body myositis (sIBM) (muscle predominant)

Earlier polymyositis was also recognized as a subtype but now the terminology has been discarded for the above-mentioned classification.

It is an autoimmune disease with unknown cause.

  • Genetic factors: The HLA 8.1 ancestral haplotype is an important risk factor in some populations.
  • Environmental factors: These include:
    • Exposure to ultraviolet (UV) light
    • Smoking
    • Infections
    • Vitamin D deficiency
    • Exposure to certain pollutants
    • Exposure to medications
    • Exposure to inhaled particles
    • Exposure to chemical agents
    • Exposure during pregnancy and infancy
  • Other factors: Statins can increase HMGCR expression in muscle, which may worsen autoimmunity against muscle tissue

  • Muscle pain or cramps
  • Muscle weakness
  • Fatigue
  • Difficulty swallowing/ breathing
  • Skin rash
  • Joint pain and swelling

Neurologist

  • Clinical examination
  • Blood tests to determine serum enzymes levels using Myositis panel which includes the auto-antibodies responsible for muscle inflammation and weakness.
  • Electromyography
  • Muscle biopsy features
  • Muscle MRI patterns

  • Corticosteroids to reduce inflammation
  • Immunosuppressive medications
  • Physiotherapy/ speech therapy
  • Management of associated systemic features
  • Early diagnosis and treatment are necessary to prevent severe weakness
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