From the Guidelines
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a distinct clinical entity from Guillain-Barré Syndrome (GBS), characterized by a chronic, relapsing-remitting or steadily progressive course, and requires ongoing immunomodulatory therapy to manage the condition and prevent permanent nerve damage. The key difference between CIDP and GBS lies in their clinical course, with GBS typically developing rapidly over days to weeks and then improving, whereas CIDP progresses more slowly over at least 8 weeks and can continue for months or years without treatment 1.
Key Characteristics of CIDP
- Chronic, relapsing-remitting or steadily progressive course
- Immune-mediated damage to the myelin sheath surrounding nerve fibers
- Requires ongoing immunomodulatory therapy to manage the condition and prevent permanent nerve damage
Treatment Options for CIDP
- Intravenous immunoglobulin (IVIG) at 2g/kg divided over 2-5 days
- Plasma exchange (PLEX)
- Corticosteroids like prednisone at 60-100mg daily with a slow taper These treatments aim to suppress the abnormal immune response causing nerve damage 1.
Comparison with GBS
Unlike GBS, which often requires only one course of treatment, CIDP patients frequently need maintenance therapy for years, with IVIG often given at 1g/kg every 3-4 weeks 1.
Importance of Early Diagnosis and Treatment
Early diagnosis and treatment are crucial to prevent permanent nerve damage and disability in CIDP patients 1.
From the Research
Definition and Relationship between CIDP and GBS
- Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a condition that can present with features of Guillain-Barré Syndrome (GBS) 2.
- GBS and CIDP are considered immune-mediated disorders with a variable duration of progression and a range in severity of weakness 3.
- The relation between GBS and CIDP is discussed, with most likely they representing parts of a continuum, arbitrarily separated by their time course 4.
Clinical Features and Treatment
- Patients with CIDP may exhibit persistent symptoms similar to those with GBS, but with a chronic course 2.
- Treatment options for CIDP include corticosteroids, intravenous immunoglobulin (IVIg), and plasma exchange (PE) 3, 4.
- Patients with apparent GBS who show persistent symptoms may benefit from corticosteroids or other treatments beneficial in the management of CIDP 2.
- In CIDP, prednisone, plasma exchange, and immune globulins are effective in a proportion of patients, with the last two being equally effective 4.
Pathogenesis and Immune Mechanisms
- The pathogenesis of inflammatory demyelinating polyneuropathies has not been elucidated yet, but involvement of the immune system has been firmly established 4.
- Preceding infections, especially with Campylobacter jejuni, and the analysis of antiganglioside antibodies lend new support to the hypothesis of molecular mimicry between epitopes on infectious agents and peripheral nerve constituents as one of the mechanisms in GBS 4.
- Immune globulins may play a role in the treatment of CIDP and GBS through idiotypic-antiidiotypic interaction, but several other mechanisms may also be involved 4.
Current and Future Therapeutic Strategies
- Current treatment options for GBS and CIDP include IVIg, PE, and corticosteroids, but a significant proportion of patients do not respond to these therapies 5.
- Research on disease pathophysiology is needed to reveal clinically and functionally relevant disease mechanisms and to develop new treatment approaches 5.
- Novel immunological and therapeutic insights may help guide therapeutic strategies in the future for GBS and CIDP 5.