Intro to Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP is caused by an autoimmune response in which the body’s immune system mistakenly attacks the myelin sheath that surrounds peripheral nerves. This leads to nerve inflammation and damage, which disrupts the signals between the nerves and muscles. The exact trigger isn’t fully understood, and there is no single known cause, though infections, genetics, or other immune system factors may play a role.

There’s no known cure yet, but many people improve significantly with treatment. Some may achieve sustained remission, while others may have ongoing symptoms or relapsing and remitting disease. Recovery varies by individual — early treatment and good response tend to improve the long-term outlook.

There are three standard or first line treatments in CIDP:

• Corticosteroids (Prednisone, Prednisolone) are similar to naturally occurring anti-inflammatory hormones made by the body, and can be used as an initial treatment. Corticosteroids often improve strength, are conveniently taken by mouth, and are inexpensive. Side effects however can limit long-term use.

• High-dose Intravenous Immune Globulins (IVIG) is the only drug that has FDA, Canadian, and European approval for treatment of CIDP. IVIG contains naturally occurring antibodies obtained from healthy volunteers. IVIG is given through a vein over the course of several hours. There are also newer treatments with preparations of higher concentrations that can be given under the skin (subcutaneous) now available for CIDP patients. See “Understanding a Self-Infused CIDP Therapy Option” for more information.

• Plasma Exchange (PE), or Plasmapheresis (PLEX), is a process by which some of the patient’s blood is removed and the blood cells returned without the liquid plasma portion of the patient’s blood. It may work by removing harmful antibodies contained in the plasma.

There are a large number of so-called second line drugs used to treat CIDP. These are used when the above standard treatments fail, cause significant side-effects, or the clinical response is not optimal.  These drugs are largely not tested in randomized controlled trials, but their use is supported by case series from the medical literature.

There are a number of so-called third line treatments, usually chemotherapy drugs, but these should be given only in selected circumstances and by those with extensive experience in their use.

There are also ongoing research studies (see https://www.gbs-cidp.org/support/resources/clinical-trials/)

Treatment of CIDP is an art. An experienced doctor is more likely to have good outcomes than someone treating their first case as is true throughout medicine. That is why we have set up the Centers of Excellence program. If treated early, most CIDP patients respond well to therapy that can limit the damage to peripheral nerves and contribute to improved function and quality of life and at times can cure the disorder altogether.

CIDP differs from GBS in that CIDP is a chronic condition with symptoms that develop over time and may relapse or go into remission, often requiring ongoing treatment, while GBS is an acute condition that typically progresses rapidly and then improves after a single episode.

The most common treatments for CIDP include intravenous immune globulin (IVIG), corticosteroids such as prednisone, and plasma exchange (plasmapheresis), with treatment plans tailored by a neurologist based on individual response.

CIDP is often chronic, so many people require ongoing or maintenance therapy to control symptoms and prevent nerve damage. The duration of treatment depends on individual response; some may be able to reduce or stop therapy over time under medical supervision.

Even with treatment, nerve healing takes time, and some nerve damage may be irreversible. Fatigue and residual weakness can persist as your nervous system recovers or adapts. Your healthcare provider may adjust therapies or recommend supportive care (e.g., therapy, exercise) to help manage these symptoms.