What is CIDP?
Marinos C. Dalakas, M.D., Principal Author
Chronic inflammatory demyelinating polyneuropathy (or polyradiculoneuropathy), abbreviated as CIDP, is the most commonly acquired demyelinating neuropathy. Since the etiology remains unknown, CIDP remains a syndrome with several variants, primarily defined by differences in clinical presentation. The classification is still debated but virtually all neurologists define the disorder as being chronic (> 8 weeks progression), demyelinating, and inflammatory or immune-mediated.
CIDP is considered to be an autoimmune disorder whereby autoantibodies attack and destroy the myelin sheath of the peripheral nerves. (Latov, 2002) It can commonly present either as a continuously progressive affliction or, less often, as a recurrent disorder in which episodes (relapsing and remittent) in individual patients may be separated by months or years.
Classic CIDP, Variant s , and GBS Similarities
The "classic" form of chronic inflammatory demyelinating polyneuropathy is characterized by symmetric proximal and distal weakness, some sensory involvement, and loss of deep tendon reflexes. However, some patients who are considered to have "variants" of CIDP present with a pure sensory disorder, some with a multifocal, asymmetric disorder, or in association with other systemic or neurologic disorders.
While the disorder is distinguished from Guillain-Barre syndrome (GBS) because of its chronic course, CIDP and GBS share many features, including:
- elevated cerebrospinal fluid (CSF) protein levels
- symmetric proximal and distal weakness
- areflexia
- demyelination
- immunopathologic features
Causes of CIDP
The cause of CIDP is not known. As a syndrome, different causes and triggers are likely. However, it is generally accepted that most, if not all, CIDP manifestations are autoimmune, triggered by infections or toxins in genetically susceptible individuals. The autoimmune nature of CIDP is suspected because of its occasional association with other immune-mediated diseases, such as systemic lupus, hepatitis B and C, HIV, and multiple sclerosis (MS), its response to immunosuppressive and immunomodulatory treatments, and pathologic findings on nerve biopsy. Other causes of neuropathy that may be difficult to distinguish from CIDP include:
- inherited neuropathy
- systemic inflammatory-autoimmune disorders
- dysproteinemias
- diabetes mellitus
- vasculitis
- other metabolic and toxic neuropathies
