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Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice
Author(s) -
Gorson Kenneth C.,
van Schaik Ivo N.,
Merkies Ingemar S. J.,
Lewis Richard A.,
Barohn Richard J.,
Koski Carol L.,
Cornblath David R.,
Hughes Richard A. C.,
Hahn Angelika F.,
Baumgarten Mona,
Goldstein Jonathan,
Katz Jonathan,
Graves Michael,
Parry Gareth,
van Doorn Pieter A.
Publication year - 2010
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1111/j.1529-8027.2010.00284.x
Subject(s) - chronic inflammatory demyelinating polyneuropathy , medicine , refractory (planetary science) , clinical trial , physical therapy , disease , clinical phenotype , clinical practice , immunology , antibody , biochemistry , chemistry , physics , astrobiology , gene , phenotype
Defining long‐term outcomes in chronic inflammatory demyelinating polyneuropathy (CIDP) has been complicated by varying definitions of treatment response and differing scales measuring impairment or disability. An expert panel was convened to devise a CIDP Disease Activity Status (CDAS) and to classify long‐term outcome by applying it to 106 patients with a consensus diagnosis of CIDP. Sixty of these cases were graded blindly by three independent reviewers to assess inter‐rater reliability. The mean duration of follow‐up was 6.4 years (range, 3 months–23 years). Eleven percent of patients were classified as cured (stable examination and off treatment for ≥5 years), 20% were in remission (stable and off treatment for <5 years), 44% had stable active disease but required ongoing therapy for at least 1 year, 7% were improving after recent initiation of therapy, and 18% had unstable active disease (treatment naïve or treatment refractory). Excellent inter‐rater reliability was observed (kappa scores: 0.93–0.97; p < 0.0001). The CDAS is considered a simple and reproducible tool to classify patients with CIDP according to disease activity and treatment status that can be applied easily in practice and potentially to select patients for clinical trials.