Rituximab for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy: A retrospective chart review (P2.4-010)

Objective: To evaluate the therapeutic response to rituximab in patients presenting with treatment-resistant CIDP. Background: Several case reports and small observational studies have demonstrated variable efficacy of rituximab for treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Design/Methods: Retrospective chart review was performed on the patients who presented to the neuromuscular clinics with CIDP and received rituximab for this diagnosis. Demographic and clinical information was obtained. Baseline and post treatment MRC sum score, inflammatory neuropathy cause and treatment (INCAT) disability score, and modified Rankin score (mRS) were retrospectively inferred from data available in the chart. Results: A total of 5 patients (2 males and 3 females; age range 36 to 72) were identified who had received rituximab for treatment of CIDP in our neuromuscular database. These have been previously treated with multiple therapies without adequate clinical response. The median number of prior therapies was three; including corticosteroids, mycophenolate mofetil, IV immune globulin, azathioprine, and plasma exchange. Three patients received rituximab for management of chronic symptoms of CIDP. Two patients received rituximab infusions while admitted for acute symptomatic exacerbation. Three of five patients demonstrated robust improvement in response to rituximab. Average MRC sum score increase was 22 (range 0–60), average decrease in INCAT was 3.2 (range 2–6), and median mRS decreased from 3 to 2 over follow up period of 9 to 24 months. Conclusions: Rituximab may be a treatment option for patients with CIDP whose symptoms are refractory to more conventional treatments. Disclosure: Dr. Varon has nothing to disclose. Dr. Pasnoor has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with TerumoBCT, Alexion Pharmaceuticals and Momenta Pharmaceuticals. Dr. Jabari has nothing to disclose. Dr. Farmakidis has nothing to disclose. Dr. Jawdat has nothing to disclose. Dr. Glenn has nothing to disclose. Dr. Statland has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Acceleron Pharma, Inc., Expansion Therapeutics, , Fulcrum Therapeutics, Genea Biocells, and Strongbridge Biopharma. Dr. Statland has received research support from FSH Society, Muscular Dystrophy Association, and the National Institutes of Health. Dr. Barohn has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with NuFACTOR Specialty Pharmacy. Dr. Barohn has received research support from Alexion, BioMarin, Cytokinetics, Eli Lilly, Ionis, Neuraltus, Novartis, PTC Therapeutics, Sanofi/Genzyme, Sarepta, Teva. Dr. Dimachkie has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alnylam, Audentes, Biomarin, Catalyst, CSL-Behring, Genzyme, Mallinckrodt, Momenta, Novartis, NuFactor, Octapharma, RMS Medical, Sanofi, Shire and Terumo. Dr. Dimachkie has received research support from Alexion, Alnylam, Amicus, Biomarin, Bristol-Myers Squibb, Catalyst, CSL-Behring, FDA/OPD, GlaxoSmithKline, Genentech, Grifols, MDA, NIH, Novartis, Genzyme, Octapharma, UCB Biopharma, Viromed and TMA.