Dominic Stone
Inflammatory muscle disease, also known as inflammatory myopathy, is a group of rare diseases that cause chronic muscle inflammation, weakness, and pain. The majority of inflammatory myopathies are autoimmune disorders, where the body's immune system attacks its own muscle fibres, blood vessels, connective tissue, organs, or joints. The four main types of inflammatory muscle disease are dermatomyositis, polymyositis, necrotizing autoimmune myositis, and inclusion-body myositis. Diagnosis of inflammatory myopathies typically involves strength exams, blood tests, electromyograms, and MRIs, and treatment options include corticosteroids, immunosuppressants, and glucocorticosteroids.
| Characteristics | Values |
|---|---|
| Type | Rare disease |
| Affects | Both children and adults |
| Main Types | Polymyositis, Dermatomyositis, Inclusion body myositis, Necrotizing autoimmune myositis |
| Symptoms | Muscle inflammation, muscle weakness, muscle pain, fatigue, difficulty sustaining physical efforts, muscle trauma, rash |
| Diagnosis | Strength exam, blood tests, electromyogram (EMG), magnetic resonance imaging (MRI), muscle biopsy |
| Treatment | Oral corticosteroids, immunosuppressants, glucocorticosteroids, methotrexate, azathioprine, intravenous immunoglobulin (IVIG), cyclosporine, tacrolimus, mycophenolate mofetil, rituximab |
| Management | Healthy diet, regular exercise, healthy weight, sun protection |
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What You'll Learn
Types of inflammatory muscle disease
Inflammatory myopathies are a group of rare diseases that involve chronic muscle inflammation, muscle weakness, and sometimes muscle pain. They are mostly considered to be autoimmune disorders, where the body's immune system attacks its own muscle fibres, blood vessels, connective tissue, organs, or joints.
The four main types of chronic inflammatory myopathy are:
Polymyositis
This type of inflammatory myopathy affects skeletal muscles on both sides of the body. It is more common in women than in men. Polymyositis is treated with high doses of prednisone or another corticosteroid drug.
Dermatomyositis
Dermatomyositis is more common in children and women and causes progressive muscle weakness. It can cause rashes, so people with this condition are advised to use sun protection. Dermatomyositis is also treated with prednisone or another corticosteroid drug.
Inclusion Body Myositis
This type of inflammatory myopathy is characterised by slow, progressive muscle weakness and muscle shrinking and loss. It can cause more involvement of the hands, resulting in dropping things or difficulty carrying heavy items. IBM has no standard treatment and is generally unresponsive to corticosteroids and immunosuppressive drugs. Most patients will need assistive devices.
Necrotising Autoimmune Myopathy
This type involves muscle weakness in the upper and lower body.
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Symptoms
Inflammatory muscle disease, also known as idiopathic inflammatory myopathy (IIM) or myositis, is a group of rare diseases that involve chronic muscle inflammation, muscle weakness, and, in some cases, muscle pain. The four main types of inflammatory muscle disease are dermatomyositis, polymyositis, necrotizing autoimmune myositis, and inclusion-body myositis.
Inflammatory muscle diseases are characterised by muscle inflammation, muscle weakness, and sometimes muscle pain. Muscle endurance can be affected, and patients often experience significant fatigue and difficulty sustaining physical efforts. About a third of patients experience significant muscle pain. Patients with inclusion body myositis may report dropping things or having difficulty carrying heavy items.
Inflammatory arthritis is another symptom that can be associated with inflammatory muscle disease. This can cause pain and swelling of the joints, generally worse with inactivity and early in the morning, and is associated with prolonged stiffness. The arthritis can resemble or overlap with rheumatoid arthritis.
Dermatomyositis, a type of inflammatory muscle disease, can cause skin rashes. People with dermatomyositis should use sun protection to prevent rashes from worsening.
Diagnostic blood tests for inflammatory muscle diseases typically show high levels of muscle enzymes, indicating muscle breakdown and damage. When cells are damaged, they leak proteins that can be found in the blood. Different types of cells have different proteins, so a spike in specific proteins can indicate which type of cell is injured.
Magnetic resonance imaging (MRI) and electromyography (EMG) tests can also be used to diagnose inflammatory muscle diseases by looking for muscle damage and measuring electrical activity in the muscles, respectively. A muscle biopsy is considered the gold standard for diagnosing myositis, as it can demonstrate inflammatory changes in the muscle tissue and rule out other causes of injury.
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Causes
Inflammatory myopathies are a group of rare diseases that involve chronic muscle inflammation, muscle weakness, and muscle pain. The cause of much inflammatory myopathy is unknown (idiopathic), and such cases are classified according to their symptoms and signs, electromyography, MRI, and laboratory findings. However, there are some known causes and associated factors.
Firstly, inflammatory myopathies are mostly considered to be autoimmune disorders, where the body's immune system attacks its own muscle fibres, blood vessels, connective tissues, organs, or joints. Myositis (inflammation of the muscle) can be caused secondary to a number of primary diseases, including infection, muscle trauma, medications and toxins, inherited muscle diseases, and other autoimmune diseases. Lupus, scleroderma, and vasculitis are autoimmune diseases that can cause muscle inflammation as a secondary feature. Polymyositis (PM), one of the main types of inflammatory myopathy, is usually an adult disease, emerging after the age of twenty, and is more common in females and Caucasians. Dermatomyositis (DM) shares these demographic tendencies, and patients with this form of myopathy should use sun protection to prevent rashes from worsening.
In addition, inflammatory myopathies may be linked to cancer, so tests to rule out cancer are often necessary. Diagnostic blood tests will usually show high levels of muscle enzymes, which indicate muscle breakdown and damage. When cells are damaged, they leak proteins that can be found in the blood, and these can also be a sign of muscle injury or disease. A muscle biopsy is often used to diagnose myositis, as it can demonstrate inflammatory changes in the muscle tissue and rule out other causes of muscle injury.
Furthermore, patients with inclusion body myositis (IBM) may experience a different pattern of weakness, with more involvement of their hands, such as dropping things or having difficulty carrying heavy items. IBM tends to emerge after the age of thirty, usually after fifty, and may be more common in males. If the criteria for PM are met but muscle weakness also affects the hands and feet or is unaccompanied by pain, IBM is suspected, and a muscle cell biopsy can confirm this.
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Diagnosis
Inflammatory myopathy, also known as idiopathic inflammatory myopathy (IIM), is a rare disease featuring muscle weakness, inflammation of muscles (myositis), and in some cases, muscle pain (myalgia). The cause of inflammatory myopathy is often unknown (idiopathic), and such cases are classified according to their symptoms and signs, electromyography, MRI, and laboratory findings.
Inflammatory myopathies are considered to be autoimmune disorders, in which the body's immune response system attacks its own muscle fibres, blood vessels, connective tissue, organs, or joints. Myositis is a type of myopathy and is the most common form of inflammatory myopathy. Myopathy is a general term that refers to diseases that affect the muscles that connect to bones (skeletal muscles).
Physical Examination
A physical examination will involve a healthcare provider examining your symptoms and asking you how it feels when you do certain movements or motions. They will look for signs of muscle weakness, pain, and fatigue.
Blood Tests
Blood tests can be used to detect raised blood serum concentrations of some muscle enzymes such as creatine kinase.
MRI (Magnetic Resonance Imaging)
An MRI can be used to detect inflammation in the muscle tissue.
EMG (Electromyography)
An EMG can detect unhealthy muscle changes.
Biopsy
A muscle biopsy is the gold standard for diagnosing myositis. It can demonstrate inflammatory changes in the muscle tissue and rule out other causes of injury to the muscle. A skin biopsy can also be helpful to determine whether a person has dermatomyositis.
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Treatment
Inflammatory muscle disease, or inflammatory myopathy, is a group of rare diseases that cause chronic muscle inflammation, muscle weakness, and pain. While there is no cure for inflammatory muscle disease, there are several treatment options available to manage symptoms and improve quality of life.
The primary goal of treating inflammatory muscle disease is to eliminate inflammation, restore muscle performance, reduce morbidity, and improve the patient's quality of life. Treatment options include medications, exercise, and lifestyle changes.
Medications used to treat inflammatory muscle disease include steroids, such as prednisone, and other corticosteroids like methylprednisolone. These medications work by suppressing the white blood cells of the immune system, which are responsible for causing damage to muscle tissue. While steroids are effective, they can have side effects, especially with long-term use. Therefore, additional medications, such as "steroid-sparing" drugs, may be prescribed to reduce the reliance on steroids. Immunosuppressive medications like methotrexate and azathioprine have also been used off-label in the treatment of inflammatory muscle disease. Acthar, a synthetic form of the hormone ACTH, is another option for treating myositis diseases.
Exercise and physical therapy play a crucial role in the treatment of inflammatory muscle disease. Regular physical activity, including aerobic exercise, can help improve muscle strength and function, increase aerobic conditioning, and enhance overall quality of life. Specific exercises, such as stretching and strengthening routines, can be recommended by healthcare providers to target affected muscle groups and improve symptoms.
Lifestyle changes can also help manage inflammatory muscle disease. Maintaining a healthy, well-balanced diet, staying at a healthy weight, and getting adequate rest are important components of treatment. Additionally, protecting the skin from sun exposure is crucial for individuals with dermatomyositis to prevent rashes from worsening. Soft or pureed foods may be recommended for those experiencing swallowing difficulties.
It is important to note that early diagnosis and treatment of inflammatory muscle disease can help prevent severe complications. Clinical trials and research studies are also ongoing, aiming to discover new and improved treatment options for these rare diseases.
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Frequently asked questions
Inflammatory muscle disease, also known as inflammatory myopathy, is a group of rare diseases that involve chronic muscle inflammation, muscle weakness, and sometimes muscle pain.
The four main types of inflammatory muscle disease are dermatomyositis, polymyositis, necrotizing autoimmune myositis, and inclusion-body myositis.
The goal of treatment is to improve daily activities and muscle strength. Suppression of immune system activity is the primary treatment strategy. The first treatment for inflammatory myopathies is usually an oral corticosteroid, such as prednisone, at a high dose.
