Rituximab in refractory chronic inflammatory demyelinating polyneuropathy

Muley, Suraj; Jacobsen, Bill; Parry, Gareth; Usman, Uzma; Ortega, Erik; Walk, David; Allen, Jeff W.; Pasnoor, Mamatha; Varon, Matthew; Dimachkie, Mazen M.

doi:10.1002/mus.26804

Cited by 50 publications

(47 citation statements)

References 27 publications

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“…This requirement for long‐term therapy underscores the need for safe, chronic immunomodulatory therapy. Newer agents, such as rituximab, hold promise in treating adult CIDP, and have been effective in case reports in pediatric patients 22,23 . Ideally, a randomized control study should be done to determine relative effectiveness of CS versus other immune modulating therapies.…”

Section: Discussionmentioning

confidence: 99%

Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy

2020

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Childhood onset chronic inflammatory demyelinating polyneuropathy (CIDP) often requires long-term immunomodulatory therapy. We report a comprehensive review of our treatment of pediatric CIDP with a focus on high-dose weekly corticosteroids ("pulse oral corticosteroids"), a treatment method that is not commonly reported. We retrospectively reviewed medical records of pediatric patients with CIDP treated at our center between 2000 and 2018 for whom we had at least 12 mo follow-up. Here, we describe the demographics, disease course, treatment regimens, and long-term outcomes of these patients. Twenty-five patients were identified for analysis. Pulse oral corticosteroid monotherapy was the predominant maintenance treatment in 56% of patients. Patients were followed for a median of 4 y. Side effects were seen in a minority of patients. The probability of a normal exam or being off treatment at last follow-up was similar regardless of predominant maintenance therapy. Pulse oral corticosteroid therapy is a safe and effective long-term treatment option in children with CIDP.

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Section: Discussionmentioning

confidence: 99%

Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy

2020

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“…Rituximab has been tried in CIDP with some case reports suggesting a favorable response [85,101]. Muley et al In a small retrospective study of 11 patients with refractory CIDP described a rapid and in many cases impressive response, indicating that rituximab may be a useful alternative to established treatments [105]. A subcutaneous efgartigimod phase II study in adults with CIDP (ADHERE trial) has recently commenced recruiting [127].…”

Section: Immune-mediated Peripheral Neuropathiesmentioning

confidence: 99%

Monoclonal Antibodies as Neurological Therapeutics

et al. 2021

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Over the last 30 years the role of monoclonal antibodies in therapeutics has increased enormously, revolutionizing treatment in most medical specialties, including neurology. Monoclonal antibodies are key therapeutic agents for several neurological conditions with diverse pathophysiological mechanisms, including multiple sclerosis, migraines and neuromuscular disease. In addition, a great number of monoclonal antibodies against several targets are being investigated for many more neurological diseases, which reflects our advances in understanding the pathogenesis of these diseases. Untangling the molecular mechanisms of disease allows monoclonal antibodies to block disease pathways accurately and efficiently with exceptional target specificity, minimizing non-specific effects. On the other hand, accumulating experience shows that monoclonal antibodies may carry class-specific and target-associated risks. This article provides an overview of different types of monoclonal antibodies and their characteristics and reviews monoclonal antibodies currently in use or under development for neurological disease.

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“…Currently, a randomized controlled trial of rituximab in patients with antineurofascin 155-positive CIDP is ongoing [69]. Rituximab may also be effective in some patients without these antibodies, especially intractable cases [70].…”

Section: Insights Into Classification and Therapeutic Strategiesmentioning

confidence: 99%

Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

Koike

Katsuno

2020

Neurol Ther

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is classically defined as polyneuropathy with symmetric involvement of the proximal and distal portions of the limbs. In addition to this ''typical CIDP'', the currently prevailing diagnostic criteria proposed by the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) define ''atypical CIDP'' as encompassing the multifocal acquired demyelinating sensory and motor (MADSAM), distal acquired demyelinating symmetric (DADS), pure sensory, pure motor, and focal subtypes. Although macrophage-induced demyelination is considered pivotal to the pathogenesis of CIDP, recent studies have indicated the presence of distinctive mechanisms initiated by autoantibodies against paranodal junction proteins, such as neurofascin 155 and contactin 1. These findings led to the emergence of the concept of nodopathy or paranodopathy. Patients with these antibodies tend to show clinical features compatible with typical CIDP or DADS, particularly the latter. In contrast, classical macrophage-induced demyelination is commonly found in some patients in each major subtype, including the typical CIDP, DADS, MADSAM, and pure sensory subtypes. Differences in the distribution of lesions and the repair processes underlying demyelination by Schwann cells may determine the differences among subtypes. In particular, the preferential involvement of proximal and distal nerve segments has been suggested to occur in typical CIDP, whereas the involvement of the middle nerve segments is conspicuous in MADSAM. These findings suggest that humoral rather than cellular immunity predominates in the former because nerve roots and neuromuscular junctions lack blood-nerve barriers. Treatment for CIDP consists of intravenous immunoglobulin (IVIg) therapy, steroids, and plasma exchange, either alone or in combination. However, patients with anti-neurofascin 155 and contactin 1 antibodies are refractory to IVIg. It has been suggested that rituximab, a monoclonal antibody to CD20, could have efficacy in these patients. Further studies are needed to validate the CIDP subtypes defined by the EFNS/PNS from the viewpoint of pathogenesis and establish therapeutic strategies based on the pathophysiologies specific to each subtype.

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Rituximab in refractory chronic inflammatory demyelinating polyneuropathy

Cited by 50 publications

References 27 publications

Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy

Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy

Monoclonal Antibodies as Neurological Therapeutics

Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

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