Other Names: CIDP, Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder that causes progressive weakness and impaired sensory function in the legs and arms. Symptoms often include tingling or numbness (first in the toes and fingers), weakness of the arms and legs, loss of deep tendon reflexes, fatigue, and abnormal sensations. Other symptoms may include pain, dysphagia, and diplopia. CIDP is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath of the peripheral nerves. CIDP is closely related to Guillain-Barre syndrome (GBS) and is considered the “chronic counterpart” of GBS. Treatment may include corticosteroids, immunosuppressant drugs, plasma exchange, physical therapy, and/or intravenous immunoglobulin (IVIG) therapy. Left untreated, 30% of people with CIDP will progress to wheelchair dependence.

 

Pathophysiology and Epidemiology

The underlying cause of CIDP is unknown. 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.

There is evidence that CIDP is related to the immune system, and that it may have multiple triggers. It is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath of the peripheral nerves. 

 

Signs and Symptoms

The most common symptoms of CIDP include: 

  • Acute demyelinating polyneuropathy
  • Areflexia
  • Absent tendon reflexes
  • Autoimmunity
  • Fatigable weakness of proximal limb muscles
  • Motor conduction block
  • Paresthesia
  • Segmental peripheral demyelination/remyelination
  • Sensory ataxia
  • Unsteady gait

Other signs and symptoms that may be present include:

  • Difficulty climbing stairs
  • Difficulty walking
  • Falls
  • Hand muscle weakness

 

Diagnosis

The diagnosis of CIDP should be considered in people with symmetric or asymmetric polyneuropathy who have progressive or relapsing-remitting symptoms for more than two months – particularly if the symptoms include positive sensory symptoms (such as tingling), proximal weakness, or absent reflexes.

The initial diagnosis of CIDP is based on signs and symptoms, but the diagnosis can be confirmed by evidence of peripheral nerve demyelination. This may be identified by either electrodiagnostic testing or by nerve biopsy. Electrodiagnostic testing is recommended for all patients with suspected CIDP. There is general agreement among the medical community that the following criteria support the diagnosis of “classic” CIDP:

  • Progression over at least two months
  • Weakness more than sensory symptoms
  • Symmetric involvement of arms and legs 
  • Proximal muscles involved along with distal muscles 
  • Reduced deep tendon reflexes throughout 
  • Increased cerebrospinal fluid protein without pleocytosis 
  • Nerve conduction evidence of a demyelinating neuropathy
  • Nerve biopsy evidence of segmental demyelination with or without inflammation

 

Management and Current Treatment

Standard treatment options for CIDP include:

  • 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 following have been approved by the FDA as orphan drugs for CIDP:

  • Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified (Brand name: Gammaked
  • Immune globulin injection [human], 10% caprylate/chromatography purified (Brand name: Gamunex-C
  • Immune Globulin Subcutaneous (Human), 20% Liquid (Brand name: Hizentra)
  • Immune globulin intravenous (human), 10% liquid (Brand name: Privigen

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

  • 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. Physical therapy may improve muscle strength, function and mobility.

 

Clinical Trials 

For a full list of clinical trials relating to CIDP, go here.

 

Resources

GBS/CIDP Foundation International 

Chronic Inflammatory Demyelinating Polyneuropathy – NORD

Chronic Inflammatory Demyelinating Polyneuropathy – NIH

 

To learn more about rare autoimmune disorders, visit our Rare Autoimmune/Auto-Inflammatory page.