🔬 Other

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Also known as: CIDP, Chronic relapsing polyneuropathy, Demyelinating polyradiculoneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare acquired immune-mediated inflammatory disorder of the peripheral nervous system. It is characterized by progressive symmetric weakness and impaired sensory function in the arms and legs. Unlike its acute counterpart, Guillain-Barré syndrome (GBS), CIDP has a chronic course, progressing for at least 8 weeks. The condition results from damage to the myelin sheath surrounding nerve fibers, leading to impaired nerve conduction and neurological dysfunction.

ICD-10: G61.81 Orphanet: 791 OMIM: 609159
👥 1-9 / 100,000 Prevalence
🔬 150 Active Trials

Imagine your body's wires (nerves) are covered in a protective coating. In CIDP, your body's defense system mistakenly attacks this coating, making it hard for signals to travel along the wires. This causes weakness and numbness, especially in your arms and legs. Doctors can give you medicine to calm down your defense system and protect the wires.

Signs & Symptoms

  • Progressive weakness in arms and legs
  • Sensory loss (numbness, tingling)
  • Loss of reflexes
  • Fatigue
  • Pain
  • Impaired balance and coordination
  • Difficulty walking
  • Muscle atrophy
  • Cranial nerve involvement (less common)

Treatment Options

MEDICATION FDA Approved

Intravenous Immunoglobulin (IVIg)

HIGHLY EFFECTIVE
THERAPY FDA Approved

Plasma Exchange (Plasmapheresis)

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Corticosteroids (e.g., Prednisone)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Subcutaneous Immunoglobulin (SCIg)

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Immunosuppressants (e.g., Azathioprine, Cyclophosphamide, Mycophenolate Mofetil)

MODERATELY EFFECTIVE
SUPPORTIVE

Physical Therapy

SUPPORTIVE
SUPPORTIVE

Occupational Therapy

SUPPORTIVE

Diagnosis

  • Clinical examination
  • Nerve conduction studies (NCS)
  • Electromyography (EMG)
  • Cerebrospinal fluid (CSF) analysis (elevated protein)
  • Nerve biopsy (in select cases)
  • MRI of the spine and brain (to rule out other conditions)

History

CIDP was first described as a distinct entity in the late 1950s and early 1960s, differentiating it from the more acute Guillain-Barré syndrome. The recognition of its chronic and relapsing nature led to the development of specific diagnostic criteria and treatment approaches.

Recent Breakthroughs

2023

Efgartigimod Approved for CIDP

Efgartigimod alfa-fcab is now approved for the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in adult patients. Efgartigimod is a human IgG1 antibody fragment that binds to the neonatal Fc receptor (FcRn), resulting in a reduction of circulating IgG.

2022

New insights into CIDP subtypes and biomarkers

Research has identified distinct CIDP subtypes based on clinical, electrophysiological, and immunological characteristics. Studies are also exploring potential biomarkers to improve diagnosis and predict treatment response.