To describe the response to rituximab in patients with treatment-resistant chronic inflammatory demyelinating polyneuropathy (CIDP) with antibodies against paranodal proteins and correlate the response with autoantibody titers.
Patients with CIDP and IgG4 anti–contactin-1 (CNTN1) or anti–neurofascin-155 (NF155) antibodies who were resistant to IV immunoglobulin and corticosteroids were treated with rituximab and followed prospectively. Immunocytochemistry was used to detect anti-CNTN1 and anti-NF155 antibodies and ELISA with human recombinant CNTN1 and NF155 proteins was used to determine antibody titers.
Two patients had a marked improvement; another patient improved slightly after 10 years of stable, severe disease; and the fourth patient had an ischemic stroke unrelated to treatment and was lost to follow-up. Autoantibodies decreased in all patients after rituximab treatment.
(1)TITLE: Blood-Based Assay for Dysferlinopathies INVENTORS (By order of signature): RH Brown, MF Ho, I Illa, E Gallardo N?: 7,172,858 PRIORITY COUNTRY: United States PRIORITY DATE: February 6th, 2007 ENTITY: Massachusetts General Hospital, Boston/ Hospital de Sant Pau, Barcelona XTENDED TO COUNTRIES: CANADA, JAPAN COMPANIES EXPLOITING IT: Athena Diagnostics, Worcester, USA
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