Chronic inflammatory demyelinating polyneuropathy

The underlying cause of CIDP is unknown. There is evidence that it is related to the immune system, and that it may have multiple triggers.^[1] It is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath of the peripheral nerves.^[2] The myelin sheath is the protective covering of nerve fibers.^[3] When myelin is damaged or removed, electrical impulses are slowed or lost, and messages transmitted from the brain are disrupted.^[4]

CIDP is not known to be inherited and is considered an acquired disorder. No clear genetic predisposition or other predisposing factors for CIDP have been identified.^[1]

Standard treatment options for CIDP include:^[3][4][5]

• intravenous immune globulin (IVIG) - adds large numbers of antibodies to the blood plasma to reduce the effect of the antibodies that are causing the problem
• glucocorticoids - help reduce inflammation and relieve symptoms
• plasma exchange - removes harmful antibodies from the blood

The choice of treatment may depend on the preference of the patient, side effects, treatment cost, duration, and availability.^[5] There are advantages and disadvantages of each treatment option:^[5]

• IVIG and plasma exchange may lead to a more rapid improvement in CIDP than glucocorticoid therapy, but are less likely than glucocorticoids to produce a remission
• IVIG is expensive, and its supply is sometimes limited
• Glucocorticoids are inexpensive, but chronic use is limited by common and important side effects
• Plasma exchange is expensive, invasive, and available only at specialized centers

Other drugs may be used when standard treatments fail or cause significant side-effects.^[4] Physical therapy may improve muscle strength, function and mobility.^[2]

The long-term outlook (prognosis) for people with CIDP appears to vary, but data regarding the prognosis is limited.^[2][5] Post-treatment life can depend on whether the disease was caught early enough to benefit from treatment options. For example, gradual onset of CIDP can delay diagnosis by several months or even years, resulting in significant nerve damage.^[4]

Complete remission, partial remission, and severe disability have all been reported.^[5] Some people may have a "bout" of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses. Some people are left with residual numbness or weakness that can lead to reduced quality of life and/or long-term care.^[2][4][6] Rare, severe outcomes that have been reported include quadriplegia, respiratory failure and death.^[6]