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Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta-analysis
Ali Al-Zuhairy1, Johannes Jakobsen1
1Department of Neurology, Copenhagen University Hospital (Rigshospitalet), Copenhagen, Denmark.
This systematic review found that 3.3% of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) died, while 8.2% remained nonambulatory. However, nearly half of CIDP patients achieved a good outcome without disability.
Area of Science:
- Neurology
- Immunology
- Clinical Epidemiology
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) outcomes vary significantly across studies.
- Uncertainty in long-term disease prognosis necessitates a comprehensive analysis.
Approach:
- A systematic review and meta-analysis was performed following PRISMA guidelines.
- Searched PubMed and EMBASE for studies with ≥2 years follow-up, published by May 2022.
- Analyzed data from 21 studies including 1199 patients, assessing case fatality, ambulation, physical function, and remission rates.
Key Points:
- Pooled case fatality rate for CIDP was 3.3% (95% CI, 1.9%–5.7%).
- 8.2% of CIDP patients were nonambulatory (95% CI, 5.7%–11.6%).
- 47.1% of patients experienced a good outcome without disability (95% CI, 39.5%–54.9%).
- The pooled remission rate was 40.8% (95% CI, 30.6%–51.8%).
Conclusions:
- CIDP has a significant impact on patient mobility and long-term function.
- While many patients achieve remission, strategies to prevent long-term impairment are needed.
- Clinical strategies should consider avoiding immunomodulating therapy in patients with established remission.
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