Rituximab‐responsive CIDP

Abstract

Chemotherapy has been used for treating chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with incosistent results. Chemotheraphy has been used for treating with inconsistent results. Rituximab is a chimeric monoclonal antibody directed against the CD20 antigen, used to treat B‐cell lymphoma and reported to be effective in some neuropathies with IgM autoreactivity. We saw a 72‐year‐old man with CIDP refractory to steroids, plasma exchange, IV Ig, and cyclosporin. He was unable to walk; severe impairment was present also at the upper limbs. Electrodiagnostic studies revealed slowing of motor and sensory conduction velocities, and prolonged distal motor latencies. Immunoelectrophoresis evidence an IgM/k monoclonal gammopathy. Antibodies to Myelin Associated Glycoprotein (MAG) and other peripheral nerve antigens were negative. Bone marrow biopsy documented a small lymphocytic B‐cell lymphoma (CD20+). Treated with Rituximab (375 mg/m2 for 4 weeks), the patient presented at follow‐up evaluations (3, 6 and 8 months later) a progressive improvement. Eventually, he was able to walk, and regained full strength at upper limbs. Consistently, electrophysiological studies improved. We suggest considering treatment with Rituximab in CIDP resistant to conventional therapy, at least in cases associated with IgM lymphoproliferative diseases.

Rituximab in chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus

“…Two recent open-label studies involving 30 patients with IgM-associated demyelinating polyneuropathy found improvement in those who were receiving treatment with RTX [16,17]. Furthermore, a positive treatment effect was reported for RTX in CIDP associated with lymphoma [6]. The clinical improvement and subsequent stabilization obtained with RTX in our patient may suggest this drug as an option in patients with CIDP and co-occurrence of DM.…”

“…In this case an alternative therapeutic approach may be beneficial. It has been reported that CIDP may respond to rituximab (RTX) [6,7]. RTX is a chimeric humanized monoclonal antibody against the CD20+ antigen on B cells [8].…”

Current treatments of chronic immune‐mediated demyelinating polyneuropathies

“…176 Individual patients with CIDP (associated with an IgM monoclonal protein, Evans syndrome, ITP, or diabetes mellitus, respectively) have been reported to show improvement with rituximab treatment. 11,25,105,142 An open-label study of rituximab in 2 patients with CIDP showed no benefit in reducing the dose of IVIg. One of the 2 patients improved, although he had more frequent relapses after rituximab and required higher doses of IVIg.…”

Chronic inflammatory demyelinating polyneuropathies: Current treatment strategies

“…Rituximab is generally well tolerated at the usual dose of 375 mg/m 2 in four weekly injections; however, side effects such as infusion-related fever, chills, nausea, hypotension, and headache as well as more serious mucocutaneous reactions are reported. In CIDP, rituximab appears to be of benefit in three case reports [80][81][82]. Rituximab has also been beneficial in anti-MAG neuropathy [83][84][85][86] and MMN associated with GM1 antibodies [85,86].…”