Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, chronic autoimmune condition that gradually damages the myelin sheath that covers the peripheral nerves. The myelin sheath protects your nerves and allows electrical signals to travel between your brain and the rest of your body.
Damage to the myelin sheath causes neurological symptoms on both sides of the body, including muscle weakness, numbness, tingling, and burning sensations that get worse over time.
UPMC neurology experts provide advanced care for CIDP, including treatment with medication, plasma exchange, or intravenous immunoglobulin therapy (IVIG).
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What Is CIDP?
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, chronic autoimmune condition that causes progressive damage to the myelin sheath that covers the peripheral nerves. The myelin sheath protects your nerves and allows electrical signals to travel between your brain and the rest of your body.
Damage to the myelin sheath causes neurological symptoms on both sides of the body, including muscle weakness, numbness, tingling, and burning sensations that get worse over time.
Is CIDP related to Guillain-Barré syndrome (GBS)?
Yes. CIDP is closely related to and causes similar symptoms to Guillain-Barré syndrome (GBS). However, GBS typically comes on quickly, rapidly worsens, and improves after a few weeks. CIDP is generally a long-term, chronic condition that gradually worsens over months or years.
What are the types of CIDP?
There are five types of CIDP, including:
- Typical CIDP (most common) — Causes muscle weakness and abnormal sensations that affect both sides of your body.
- Multifocal CIDP or Lewis-Sumner syndrome — Causes abnormal sensations and muscle weakness that only affect specific parts of your body.
- Distal CIDP — Causes muscle weakness, abnormal sensations, and loss of reflexes in the lower legs and the hands may be affected.
- Pure motor CIDP — Causes muscle weakness and loss of reflexes on both sides of your body, but no abnormal sensations.
- Pure sensory CIDP — Causes abnormal sensations, along with balance issues and difficulty walking.
How common is CIDP?
CIDP is difficult to diagnose, making it hard to determine how many people have the condition. Researchers estimate that CIDP affects between one and nine individuals per 100,000 in the U.S. each year.
What causes CIDP?
CIDP occurs when your immune system mistakenly attacks the myelin sheath that covers your nerves, causing damage that prevents your nerves from transmitting electrical signals to and from your brain. However, researchers aren’t sure what triggers the condition.
CIDP risk factors
There are no known risk factors for CIDP. CIDP can affect people of all ages.
Complications of CIDP
Complications of CIDP include:
- Disability.
- Increased risk of infection due to treatments that suppress your immune system.
- Permanent nerve damage.
- Relapse of symptoms.
How can I prevent CIDP?
There is nothing you can do to prevent CIDP. However, getting early treatment may reduce your risk of developing serious complications.
What Are the Signs and Symptoms of CIDP?
Symptoms of CIDP vary depending on the type you have, but typically affect your:
- Feet
- Hands
- Hips
- Shoulders
- Thighs
- Upper arms
The main symptom is muscle weakness that gets progressively worse over at least eight weeks. Symptoms may start slowly, appear suddenly, or come and go over time.
Other symptoms may include:
- Balance and mobility problems.
- Difficulty swallowing.
- Double vision.
- Loss of coordination.
- Loss of muscle mass.
- Loss of reflexes.
- Pain.
- Sensations such as tingling, numbness, or burning in your fingers or toes. Pain is less common in CIDP compared to some other neuropathies, like diabetic neuropathy.
When should I see a doctor about my CIDP symptoms?
If you have symptoms of CIDP, you should schedule an appointment right away. If symptoms are severe and affect your ability to breathe — a rare complication — dial 911 or go to your nearest hospital Emergency Department.
Early diagnosis and treatment combined with ongoing care may reduce your risk of permanent damage and other complications.
How Do You Diagnose CIDP?
To diagnose CIDP, your doctor will perform physical and neurological examinations and review your symptoms and medical history. Your doctor may also order tests to confirm your diagnosis or rule out other problems.
What to expect during your visit
During your visit, your doctor will:
- Ask you about your symptoms.
- Order tests.
- Perform a physical and neurological exam.
- Review your medical history.
Tests to diagnose CIDP
Tests to diagnose CIDP include:
- Neurological examination — A noninvasive test to check your reflexes, balance, strength, and movement.
- Electromyography (EMG)/Nerve conduction study (NCS) — Measures the speed of electrical activity passing through your nerves and the electrical activity in your muscles. When performed appropriately, this is the best test for CIDP.
- Blood tests — Checks for antibodies linked to CIDP and rules out other conditions.
- Cerebrospinal fluid analysis (spinal tap or lumbar puncture) — Involves inserting a needle into your lower back to collect a sample of cerebrospinal fluid (CSF) for analysis in a lab. This test is done infrequently.
- Magnetic resonance imaging (MRI) — Creates images of your spine using contrast dye to look for damaged or abnormal nerve roots.
- Nerve biopsy — A procedure to take a sample of one of your nerves to look for damage to the myelin sheath.
CIDP prognosis
Without treatment, CIDP can lead to permanent nerve damage that may cause disability. With treatment, CIDP symptoms improve in about nine out of 10 people diagnosed.
However, symptom recurrence is common, affecting about half of those diagnosed. People diagnosed at a younger age tend to have better treatment outcomes than those diagnosed later in life.
What is the life expectancy of someone with CIDP?
CIDP is not life-threatening and does not affect life expectancy. Most people with CIDP have an average life expectancy.
How Do You Treat CIDP?
The goals of CIDP treatment are to improve symptoms and reduce the risk of recurrence and permanent damage.
Treatment options may include:
Medications
Your doctor may prescribe medications such as corticosteroids to manage myelin damage and suppress your immune system. Other drugs may also be used to treat symptoms.
If symptoms are severe, you may receive medications through an IV. If you are taking medications by mouth, you may start by taking a higher dose that is lowered as your symptoms improve. In some cases, you may need to continue taking medications on a long-term basis to control symptoms or prevent recurrence.
Plasma exchange (plasmapheresis)
Plasmapheresis involves using a machine to separate the liquid part of your blood, called plasma, from your blood cells. Your plasma is then filtered to remove the antibodies that are causing nerve damage before being returned to your body.
Because CIDP is a long-term condition with high recurrence rates, you may need to have plasmapheresis treatments regularly to manage symptoms.
Intravenous immunoglobulin therapy (IVIG)
During IVIG, you will receive an intravenous injection of donor immunoglobulins. Immunoglobulins are a natural part of your immune system and protect your body by attacking foreign organisms.
Immunoglobulins may prevent your immune system from further damaging your nerves. Depending on your condition, you may need to have regular IVIG treatments to manage symptoms.
Clinical trials
You may be eligible to participate in a clinical trial studying new treatments for CIDP. Your doctor will let you know if you qualify for a clinical trial.
Why Choose UPMC for CIDP Care?
When you choose UPMC for CIDP care, you will receive:
- Multidisciplinary care — Our team of neurologists and autoimmune disease experts will work together to develop a customized treatment plan that helps you manage symptoms, reduce your risk of complications, and minimize treatment side effects.
- Access to advanced clinical trials — Our team participates in national, multicenter clinical trials evaluating new treatment options, allowing you to contribute to research advancing care for autoimmune disorders.
- Seamless, ongoing treatment — If you are diagnosed with CIDP, our team will develop a plan for your follow-up care, including ongoing treatment as needed.
