Inflammatory Myopathies
Introduction
Inflammatory myopathies are a group of rare diseases that cause muscle inflammation and weakness. They are autoimmune diseases. Common types include polymyositis, dermatomyositis and Inclusion body myositis.
What are the features of Inflammatory Myopathies?
- Polymyositis is a rare disease
- Ocular and facial muscle weakness present in polymyositis
- Dermatomyositis occurs in children and adults
- Inclusion body myositis (IBM) present in 30-to-40 year of age
- Serum creatine phosphokinase (CPK) level may be normal
Bohan & Peter Diagnostic Criteria For Dermatomyositis / Polymyositis
- Symmetric proximal muscle weakness
- Elevated muscle enzyme creatine phosphokinase (CPK) levels
- A myopathic pattern on electromyogram (EMG)
- Evidence of an endomysial, mononuclear inflammatory infiltrate on muscle biopsy
- Characteristic skin rash of dermatomyositis (Heliotrope rash or Gottron’s papules)
- All first four criteria must be present
- Characteristic rash along with three other criteria must be present
Polymyositis
- It is a rare disease.
- Incidence: Less than 25 per 100000 population
- Age: It usually develops between the ages of 30-to-60.
- Facial and extraocular muscle involvement is not a characteristic of polymyositis.
Dermatomyositis
- Dermatomyositis can occur in both children and adults.
- Children: Between the ages of 5 and 15.
- Adults: Between the ages of 40 and 60.
Inclusion Body Myositis
- It mostly occurs over 50 years of age.
Answer(s)
- Polymyositis is a rare disease-True
- Ocular and facial muscle weakness present in polymyositis-False
- Dermatomyositis occurs in children and adults-True
- Inclusion body myositis (IBM) present in 30-to-40 year of age-False
- Serum creatine phosphokinase (CPK) level may be normal-False
Bonus Pearls:
Do you know! characteristic skin rash is must to diagnose dermatomyositis.
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