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ID: 101
Category: Autoimmune Disease
CreatedBy: 1
UpdatedBy: 1
createdon: 10 Dec 2022
updatedon: 17 May 2023

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Author: Khoa Tran
Published Dec 10, 2022
Updated May 17, 2023

Table of contents

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Etymology and Pronunciation

Inflammatory (in-flam-uh-tawr-ee or in-flam-uh-toh-ree)
inflammare - Latin means "to set on fire"

Demyelinating (dee-my-uh-luh-ney-ting)
de- - Latin for "deprive", "from, away from, off"
myelos - Greek for "marrow" or "spinal cord"

Polyneuropathy (pol-ee-noo-rop-uh-thee)
poly - Greek for "many"
neuron - Greek for "nerve"
pathy/pathos - Greek for "suffering" or "disease"

History of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

The earliest description of CIDP-like symptoms was published in 1958 by a French neurologist named Jean-Martin Charcot. He described a patient with progressive weakness and loss of sensation in the limbs, which he attributed to a chronic inflammation of the peripheral nerves. However, it wasn't until the 1970s that the disease was recognized as a distinct entity.

In 1975, Peter O. Behan and his colleagues in Glasgow, Scotland, described a series of patients with a chronic inflammatory disorder that affected the peripheral nerves. They coined the term "chronic inflammatory demyelinating polyneuropathy" to describe the disease, which they found to be similar to Guillain-Barré syndrome, another autoimmune disorder that affects the peripheral nerves but usually resolves within weeks to months.

Around the same time, Dr. Peter Thomas and his team in London, England, also described a series of patients with a similar chronic inflammatory disorder affecting the peripheral nerves. They independently coined the term "chronic relapsing polyneuropathy" to describe the disease.

Over the years, researchers have made significant progress in understanding the pathophysiology of CIDP. It is now known to be caused by an autoimmune response that targets the myelin sheath surrounding the nerves, leading to demyelination and nerve dysfunction. The disease is diagnosed based on clinical features and supported by laboratory tests, such as nerve conduction studies and electromyography.

In the 1990s, treatment options for CIDP were limited, and patients often had poor outcomes. However, with the introduction of intravenous immunoglobulin (IVIg) and corticosteroids, the prognosis for CIDP patients has improved significantly. Today, researchers continue to investigate the underlying mechanisms of the disease and develop new treatments to improve outcomes for patients with CIDP.

Modern Understanding of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a chronic condition that affects the peripheral nervous system and causes progressive weakness and sensory loss. It is considered a type of autoimmune disorder, where the body's immune system mistakenly attacks its own tissues, in this case, the myelin that surrounds the nerves.

Myelin is a protective sheath that surrounds the nerves and helps them conduct electrical signals. In CIDP, the immune system attacks the myelin, leading to inflammation and damage, which interferes with nerve conduction. As a result, individuals with CIDP experience progressive weakness, sensory loss, and other symptoms that can affect their ability to perform everyday tasks.

It is considered a variant of Guillain-Barré syndrome (GBS), an immune-mediated disorder in which the immune system mistakenly attacks the nerve fibers. However, CIDP is a chronic condition that develops over a longer period of time and can persist for years, while Guillain-Barré syndrome is usually a short-term condition that improves with treatment.

The diagnosis of CIDP is based on the clinical presentation, nerve conduction studies, and nerve biopsy. Treatment options for CIDP include immunomodulatory therapies such as intravenous immunoglobulin (IVIg) and plasma exchange, as well as corticosteroids and other immunosuppressive drugs. In some cases, physical therapy and occupational therapy may also be recommended to help improve muscle strength and coordination. Early diagnosis and aggressive treatment are important for improving outcomes in patients with CIDP. If you suspect that you have CIDP, it is important to seek medical attention as soon as possible to ensure proper diagnosis and treatment.

Causes of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

The exact cause of CIDP is unknown, but it is believed to occur when the immune system mistakenly attacks and damages the myelin sheath, which is the protective covering that surrounds the nerves and helps to conduct nerve impulses.

It is thought that genetic factors, environmental triggers, and viral or bacterial infections may play a role in the development of CIDP. In some cases, CIDP can occur as a result of an abnormal response to a vaccination or medication. In rare cases, it can also occur in people with certain types of cancer, such as lymphoma.

Certain risk factors may also increase the likelihood of developing CIDP, such as age (most commonly diagnosed in people over age 50), being male, and having a history of autoimmune disorders or other chronic diseases.

Treatments for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

While there is no cure for CIDP, several treatment options are available to help manage symptoms and slow disease progression.

- Intravenous Immunoglobulin (IVIG): This is the most common and effective treatment for CIDP. IVIG is an infusion of human antibodies that help regulate the immune system and reduce inflammation. This treatment can help improve strength and reduce symptoms in about 70% of patients.
- Corticosteroids: These are powerful anti-inflammatory drugs that can help reduce nerve inflammation and improve symptoms. Prednisone is the most commonly used corticosteroid for CIDP treatment. However, prolonged use can lead to side effects such as weight gain, mood changes, and increased risk of infection.
- Plasmapheresis: This is a procedure that removes the harmful antibodies from the bloodstream. During plasmapheresis, blood is drawn from the patient, and plasma (the liquid component of blood containing the antibodies) is separated from the red and white blood cells. The blood cells are then returned to the patient, and the plasma is replaced with a substitute. Plasmapheresis can provide temporary relief of symptoms, but the effects are usually short-lived.
- Immunosuppressants: These drugs suppress the immune system and reduce inflammation. Examples of immunosuppressants used to treat CIDP include azathioprine, mycophenolate mofetil, and methotrexate. These medications can have significant side effects and are usually reserved for patients who do not respond to other treatments.
- Monoclonal Antibodies: These drugs target specific components of the immune system that are responsible for inflammation. Rituximab, a monoclonal antibody that targets B cells, has shown promising results in treating CIDP in some patients.
- Physical therapy: Physical therapy can help improve muscle strength and coordination, as well as prevent muscle atrophy and contractures. It is an essential part of the treatment plan for CIDP and can help improve quality of life.
- Occupational therapy: Occupational therapy can help patients learn new ways to perform daily activities and improve their ability to perform self-care tasks.
- Pain management: Pain is a common symptom of CIDP, and pain management strategies such as analgesics, neuropathic pain medications, and nerve blocks can be effective in relieving pain.

Treatment for CIDP is usually individualized based on the severity of symptoms and response to treatment. A combination of the above treatments may be necessary to manage CIDP effectively.

Lifestyle Changes

While lifestyle changes cannot cure CIDP, they can help manage symptoms and improve overall well-being. Here are some lifestyle changes that may be beneficial:

- Follow treatment plan: Work closely with your healthcare provider to develop an effective treatment plan for CIDP. This may include medications, such as corticosteroids or immunosuppressants, and regular medical check-ups. Adhering to the prescribed treatment plan is crucial for managing symptoms and preventing disease progression.
- Engage in physical therapy: Physical therapy can be helpful in managing CIDP symptoms. A physical therapist can design a customized exercise program to improve strength, balance, and mobility. Regular exercise can also promote overall well-being and reduce the risk of muscle atrophy.
- Practice stress management: Chronic illnesses like CIDP can be physically and emotionally demanding. It is important to manage stress effectively. Explore stress reduction techniques such as deep breathing exercises, meditation, yoga, mindfulness, and engaging in activities that bring you joy and relaxation.
- Maintain a balanced diet: Eating a balanced diet can support overall health and well-being. Focus on consuming a variety of fruits, vegetables, whole grains, lean proteins, and healthy fats. Adequate nutrition can support immune function, provide essential nutrients, and promote optimal nerve health.
- Monitor and manage pain: CIDP can cause pain and discomfort. Discuss pain management strategies with your healthcare provider, which may include medications, physical therapy, and alternative therapies like acupuncture or transcutaneous electrical nerve stimulation (TENS).
- Adapt your living environment: Make necessary modifications to your living environment to enhance safety and accessibility. This may include installing handrails, using assistive devices, or rearranging furniture to minimize fall risks.
- Stay informed and educated: Stay updated with the latest research and information about CIDP. Educate yourself about the condition, treatment options, and potential lifestyle modifications that may be beneficial. This knowledge can empower you to actively participate in your own care.

Symptoms

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Muscle Weakness
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Numbness in fingers and toes

Confirmation Tests

- Electromyography (EMG)
- Nerve conduction test
- Spinal fluid analysis
- Nerve biopsy
- Blood test

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