Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal

J Neurol Neurosurg Psychiatry. 2014 Aug;85(8):901-6. doi: 10.1136/jnnp-2013-306105. Epub 2013 Dec 5.

Abstract

Background: About 40% of responders to treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remain treatment dependent and have a relapse if treatment is interrupted.

Objective: To look for factors associated with treatment dependence or successful withdrawal in CIDP patients.

Methods: We retrospectively studied 70 responder CIDP patients comprising 34 patients who remained treatment dependent (treatment-dependent group) and 36 patients whose treatment could be discontinued (treatment withdrawal group). Clinical, biological, electrophysiological and therapeutic features were compared between these groups.

Results: A multifocal deficit was more frequent in the treatment-dependent group (35%) than in the treatment withdrawal group (8%) (p<0.01). The most frequent effective treatment was intravenous immunoglobulin (IVIG) for the treatment-dependent group (79%). In this group, more patients were resistant to corticosteroids in first-line therapy (93%) than in the treatment withdrawal group (40%) (p=0.002). The delay to effective treatment was significantly shorter for the treatment withdrawal group than for the treatment-dependent group (mean 11.1 vs 31.2 months; p<0.01). The rate of successful withdrawal was lower with IVIG (29%) than with corticosteroids (83%) (p<0.001).

Conclusions: When compared with the treatment withdrawal group, the treatment-dependent group was more frequently responsive to IVIG, more frequently resistant to corticosteroids in first-line treatment, had a longer delay to effective treatment and was more likely to present a multifocal deficit. The rate of successful withdrawal seems to be higher with corticosteroids, but a prospective study with a long-term follow-up is needed to confirm these features.

Keywords: NEUROPATHY.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Age Factors
  • Anti-Inflammatory Agents / therapeutic use
  • Disease Progression
  • Electrodiagnosis
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy*
  • Recurrence
  • Retrospective Studies
  • Sex Factors
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents
  • Immunoglobulins, Intravenous