title: Disease activity in chronic inflammatory demyelinating polyneuropathy.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Neurology, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia. Electronic address: hana.albulaihe@gmail.com.
authors.forename: Hana
authors.initials: H
authors.lastname: Albulaihe
authors.affiliation: Department of Neurology, King Fahad Hospital of the University, University of Dammam, Dammam, Saudi Arabia. Electronic address: dr_majed50@hotmail.com.
authors.forename: Majed
authors.initials: M
authors.lastname: Alabdali
authors.affiliation: Department of Neurology, King Fahad Hospital of the University, University of Dammam, Dammam, Saudi Arabia. Electronic address: dr.aaalsulaiman@gmail.com.
authors.forename: Abdulla
authors.initials: A
authors.lastname: Alsulaiman
authors.affiliation: Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. Electronic address: alonabmail@gmail.com.
authors.forename: Alon
authors.initials: A
authors.lastname: Abraham
authors.affiliation: Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. Electronic address: ari.breiner@uhn.ca.
authors.forename: Ari
authors.initials: A
authors.lastname: Breiner
authors.affiliation: Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. Electronic address: c.barnetttapia@mail.utoronto.ca.
authors.forename: Carolina
authors.initials: C
authors.lastname: Barnett
authors.affiliation: Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. Electronic address: hans.katzberg@utoronto.ca.
authors.forename: Hans D
authors.initials: HD
authors.lastname: Katzberg
authors.affiliation: Division of Endocrinology and Metabolism, Department of Medicine, Mount Sinai, Hospital and Lunenfeld Tanenbaum Research Institute, University of Toronto, Toronto, Canada. Electronic address: lovblom@lunenfeld.ca.
authors.forename: Leif E
authors.initials: LE
authors.lastname: Lovblom
authors.affiliation: Division of Endocrinology and Metabolism, Department of Medicine, Mount Sinai, Hospital and Lunenfeld Tanenbaum Research Institute, University of Toronto, Toronto, Canada. Electronic address: bperkins@mtsinai.on.ca.
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Perkins
authors.affiliation: Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Division of Neurology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. Electronic address: vera.bril@utoronto.ca.
authors.forename: Vera
authors.initials: V
authors.lastname: Bril
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000145:classification / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy*; D012684:Sensory Thresholds; D014732:Vibration
keywords: Activity; Chronic inflammatory demyelinating polyneuropathy; Disease; Neuropathy; Scale