From the Guidelines
Approximately 5% of patients with Guillain-Barré Syndrome (GBS) develop Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), typically characterized by repeated clinical relapses suggesting a more chronic disease process 1.
Key Considerations
- The transition from GBS to CIDP is crucial to identify, as CIDP requires long-term immunomodulatory treatment, unlike the typically monophasic GBS.
- Patients with GBS who experience symptoms beyond 8-12 weeks, worsening after initial improvement, or multiple relapses should be evaluated for CIDP.
- The pathophysiology of both conditions involves immune-mediated damage to peripheral nerves, but CIDP represents a chronic form with ongoing inflammation.
Diagnostic and Treatment Implications
- Electrophysiological studies and cerebrospinal fluid examinations are essential for diagnosing GBS and distinguishing it from CIDP.
- Treatment approaches differ significantly between GBS and CIDP, with CIDP often requiring long-term immunoglobulin therapy, corticosteroids, or other immunosuppressants.
- The diagnosis and management of GBS can be challenging due to heterogeneity in clinical presentation and the lack of highly sensitive and specific diagnostic tools or biomarkers 1.
Clinical Guidance
- A multidisciplinary approach, including supportive medical care and immunotherapy, is necessary for managing GBS.
- Intravenous immunoglobulin and plasma exchange are equally effective in treating GBS, but their efficacy in CIDP may differ 1.
- Clinical improvement in GBS is usually most extensive in the first year after disease onset and can continue for more than 5 years 1.
From the Research
Guillain-Barré Syndrome and CIDP
- Guillain-Barré syndrome (GBS) is an acute polyneuropathy with a variable degree of weakness that reaches its maximal severity within 4 weeks 2.
- About 5% of patients initially diagnosed with GBS turn out to have chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with acute onset (A-CIDP) 2.
- A study found that 11 out of 663 patients (2%) with GBS were later diagnosed as CIDP, and these patients had a slow progression or relapse beyond 8 weeks 3.
Diagnosis and Treatment
- The diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology 4.
- Intravenous immunoglobulin and plasma exchange remain the primary modalities of treatment for GBS, regardless of the electrophysiological subtype 4.
- Patients with CIDP may respond to prednisone, plasma exchange, or immune globulins, and switching therapy may be effective due to the chronic course of the disease 5.
Percentage of GBS Patients Developing CIDP
- Approximately 5% of GBS patients develop CIDP with acute onset (A-CIDP) 2.
- Another study found that 2% of GBS patients were later diagnosed as CIDP 3.
- These studies suggest that a small percentage of GBS patients may develop CIDP, but the exact percentage may vary depending on the study and population 2, 3.