Background Intravenous immunoglobulin is a first line treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). ABN guidelines have highlighted the need for evidence of IVIg efficacy in pwCIDP using validated instruments.
Aim 1) To evaluate evidence on CIDP efficacy collected on pwCIDP admitted to the Royal Devon and Exeter Hospital for IVIg. 2) Based on (1), to develop an assessment tool for measuring the efficacy of IVIg therapy in pwCIDP.
Methods 1) Retrospective evaluation of data from admissions to the RD&E (January-June 2013). 2) Identification of candidate validated instruments in CIDP, scale development and prospective application.
Results Clinical entries (N=24) proved widely variable and generally poor (33% no neurological assessment; 29% individual muscle scores available; 39% generic comments only). We subsequently developed a composite of CIDP scales addressing strength (34-item MRC summed score), sensation (INCAT sensory summed score) and activity limitations (Overall Neuropathy Limitations Scale), applying it prospectively (August-December 2013). Analysis of the dataset (N=11) suggests a wide degree of involvement for each variable (mean MRC summed score 127/170, SD 41; mean INCAT score 13/64, SD 17; mean ONLS 5.2/12, SD 2.8) in this small cohort.
Conclusion Our assessment tool captures relevant aspects of disease impact in pwCIDP and may help support responsible use of IVIg and disease monitoring.
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