Disease activity in chronic inflammatory demyelinating polyneuropathy

Hana Albulaihe, Majed Alabdali, Abdulla Alsulaiman, Alon Abraham, Ari Breiner, Carolina Barnett, Hans D. Katzberg, Leif E. Lovblom, Bruce A. Perkins, Vera Bril*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

Introduction Evaluation of disease status in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) is often done by a combination of clinical evaluation and electrodiagnostic studies. A CIDP disease activity status (CDAS) was developed to standardize outcomes in CIDP patients. We aimed to determine if the CDAS was concordant with classical evaluation and whether CDAS enables benchmarking of CIDP. Methods We performed a retrospective chart review of 305 CIDP patients and identified 206 patients with > 1 visit and applied the CDAS to this cohort. We examined relationships between the CDAS and classical evaluation as to outcomes and compared our cohort to other CIDP cohorts who had CDAS. Results We found that the CDAS mirrored disease severity as measured by electrophysiology and vibration perception thresholds in that CDAS class 5 had more severe neuropathy. Our results are similar to other cohorts in the middle CDAS strata with the exception of fewer subjects in CDAS 1 and more in CDAS 5. The only demographic factor predicting CDAS 5 in our cohort was age, and the overall treatment response rate using the CDAS classification was 79.3%. Conclusions CDAS appears to have sufficient face-validity as a grading system to assess disease activity in relation to treatment status. The use of CDAS appears to allow benchmarking of patients with CIDP that may be useful in subject selection for clinical trials and also to highlight differences in practice.

Original languageEnglish
Pages (from-to)204-209
Number of pages6
JournalJournal of the Neurological Sciences
Volume369
DOIs
StatePublished - 15 Oct 2016
Externally publishedYes

Keywords

  • Activity
  • Chronic inflammatory demyelinating polyneuropathy
  • Disease
  • Neuropathy
  • Scale

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