Inflammatory Myopathy (IFM)
Inflammatory Myopathy (IFM)
Inflammatory Myopathy (IFM) is a group of chronic autoimmune disorders characterized by muscle inflammation and weakness. They are often referred to as idiopathic inflammatory myopathies (IIMs) because their exact cause is unknown. The primary types include polymyositis, dermatomyositis, and inclusion body myositis (IBM).
Types of Inflammatory Myopathies
- Polymyositis (PM):
Affects muscles closest to the trunk of the body, such as those in the shoulders, hips, and thighs. Symptoms include progressive muscle weakness and fatigue. It is more common in women than men. - Dermatomyositis (DM):
Similar to polymyositis but also involves characteristic skin rashes. The rash can appear on the eyelids, knuckles, knees, or elbows. Dermatomyositis is more common in children and women. - Inclusion Body Myositis (IBM):
The most common type in older adults, typically affecting the muscles of the wrists and fingers and the front of the thighs. It progresses slowly and can cause difficulty with swallowing and walking.
Symptoms
- Muscle weakness, particularly in proximal muscles (those closest to the trunk of the body).
- Difficulty with activities like climbing stairs, lifting objects, or getting up from a seated position.
- Fatigue and muscle pain.
- Swallowing or breathing difficulties in some cases.
- Skin rashes in dermatomyositis, which may include heliotrope rash (purple rash around the eyes) or Gottron’s papules (flat-topped, pink or purple lesions on the knuckles).
Causes and Risk Factors
- The exact cause is unknown, but they are believed to be autoimmune disorders, possibly triggered by infections, toxins, or other environmental factors.
- There is a higher incidence in women than men.
- Dermatomyositis is more common in children, while polymyositis and IBM are more common in adults.
Diagnosis
- Physical examination and medical history.
- Blood tests to check for elevated muscle enzymes like creatine kinase.
- Electromyography (EMG) to assess muscle electrical activity.
- Magnetic Resonance Imaging (MRI) to visualize muscle damage.
- Muscle biopsy to confirm inflammation and muscle damage.
Treatment
- Initial Treatment: High-dose oral corticosteroids (e.g., prednisone) to reduce inflammation.
- Long-term Management: Immunosuppressive drugs like methotrexate or azathioprine to maintain disease control and minimize steroid side effects.
- Additional Therapies: Intravenous immunoglobulin (IVIG) or rituximab for severe cases.
- Supportive Care: Physical therapy to maintain muscle strength and function.
Summary Table
| Feature | Details |
|---|---|
| Types | Polymyositis, dermatomyositis, inclusion body myositis (IBM) |
| Symptoms | Muscle weakness, fatigue, difficulty swallowing or breathing, skin rashes (in DM) |
| Causes | Unknown; believed to be autoimmune, possibly triggered by infections or toxins |
| Diagnosis | Physical exam, blood tests, EMG, MRI, muscle biopsy |
| Treatment | Corticosteroids, immunosuppressants, IVIG, physical therapy |
| Complications | Swallowing difficulties, respiratory issues, potential association with cancer |
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References
- American College of Rheumatology: Inflammatory Myopathies https://rheumatology.org/patients/inflammatory-myopathies
- BrainFacts: Inflammatory Myopathies https://www.brainfacts.org/diseases-and-disorders/neurological-disorders-az/diseases-a-to-z-from-ninds/inflammatory-myopathies
- MedlinePlus Genetics: Idiopathic Inflammatory Myopathy https://medlineplus.gov/genetics/condition/idiopathic-inflammatory-myopathy/
- NYU Langone Health: Diagnosing Inflammatory Myopathies https://nyulangone.org/conditions/inflammatory-myopathies/diagnosis
- Mayo Clinic: Polymyositis https://www.mayoclinic.org/diseases-conditions/polymyositis/symptoms-causes/syc-20353208
- Wikipedia: Inflammatory Myopathy https://en.wikipedia.org/wiki/Inflammatory_myopathy
Inflammatory myopathies are chronic conditions requiring ongoing management to control symptoms and prevent complications. Early diagnosis and treatment can significantly improve quality of life for affected individuals.
