Rituximab Responsive Relapsing–Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review

Remiche, Gauthier; Monteiro, Marta Lamartine S.; Catalano, Concetta; Hougardy, Jean-Michel; Delmont, Émilien; Boucraut, José; Mavroudakis, Nicolas

doi:10.1097/cnd.0000000000000395

Cited by 3 publications

(2 citation statements)

References 20 publications

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“…Conversely, recent case reports have demonstrated favorable outcomes in patients with AN treated with rituximab, suggesting potential therapeutic strategies for this subgroup. [59][60][61][62][63][64] Given the pathogenic role of IgG4 autoantibodies in AN, long-term autoantibody depletion emerges as a promising therapeutic approach. Rituximab, which targets CD20-positive cells, depletes B cells from the pro-B to memory B-cell stages, precursors to plasma cells, and exerts long-term suppressive effects on autoantibody production.…”

Section: Treatment Strategies For An With Igg4 Autoantibodiesmentioning

confidence: 99%

Section: Treatment Strategies For An With Igg4 Autoantibodiesmentioning

confidence: 99%

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Autoimmune nodopathy

Iijima

2024

Clinical & Exp Neuroim

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Autoimmune nodopathy (AN) is characterized by the presence of autoantibodies targeting molecules essential for saltatory conduction in myelinated nerves. Clinical manifestations of AN show similarities with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including progressive and symmetrical sensorimotor deficits with electrophysiological demyelinating features in nerve conduction studies. Although no common autoantibodies have yet been identified in CIDP, AN is characterized by autoantibodies that primarily target specific molecular complex structures represented by the Ranvier node and paranode. Furthermore, these autoantibodies, such as neurofascin‐155 (NF155), contactin‐1 (CNTN1), contactin‐related protein 1 (Caspr1), and the CNTN1/Caspr1 complex, are illustrative examples of such autoantibodies. They can disrupt the septal‐like junction of paranodes without triggering cellular immune responses. AN manifests uniquely with symptoms such as ataxia, tremors, and markedly high cerebrospinal fluid (CSF) protein levels, often accompanied by nerve root and cranial nerve hypertrophy. Notably, this condition is resistant to immunotherapies typically effective against CIDP, including intravenous immunoglobulin therapy (IVIg). Current evidence suggests that B‐cell depletion therapies, such as rituximab, could benefit AN treatment. Since it has been suggested that existing treatments for CIDP may be effective in cases of autoantibody positivity of subclasses other than IgG4, CIDP that is resistant to conventional therapy requires novel therapeutic strategies that take into account the possibility of IgG4 autoantibodies.

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Section: Treatment Strategies For An With Igg4 Autoantibodiesmentioning

confidence: 99%

Section: Treatment Strategies For An With Igg4 Autoantibodiesmentioning

confidence: 99%

Autoimmune nodopathy

Iijima

2024

Clinical & Exp Neuroim

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Characteristics of anti-contactin1 antibody positive autoimmune nodopathies combined with membranous nephropathy

Liu,

Yang,

Zhao

et al. 2024

Journal of Neuroimmunology

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Effect of low-dose rituximab treatment on autoimmune nodopathy with anti-contactin 1 antibody

Hou

Zhang²,

Yu³

et al. 2022

Front. Immunol.

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BackgroundAutoimmune nodopathy with anti-contactin-1 (CNTN1) responds well to rituximab instead of traditional therapies. Although a low-dose rituximab regimen was administered to patients with other autoimmune diseases, such as myasthenia gravis and neuromyelitis optica spectrum disorders, and satisfactory outcomes were obtained, this low-dose rituximab regimen has not been trialed in anti-CNTN1-positive patients.MethodsAnti–CNTN1 nodopathy patients were enrolled in this prospective, open-label, self-controlled pilot study. A cell-based assay was used to detect anti-CNTN1 antibodies and their subclasses in both serum and cerebrospinal fluid. Clinical features were evaluated at baseline, 2 days, 14 days, and 6 months after single low-dose rituximab treatment (600 mg). The titers of the subclasses of anti-CNTN1 antibody and peripheral B cells were also evaluated at baseline, 2 days, and 6 months after the rituximab regimen.ResultsTwo patients with anti–CNTN1 antibodies were enrolled. Both patients had neurological symptoms including muscle weakness, tremor, sensory ataxia, numbness and mild nephrotic symptoms. In the field of neurological symptoms, sensory ataxia markedly improved, and the titer of anti-CNTN1 antibody as well as CD19+ B cells decreased only two days following low-dose rituximab treatment. Other neurological symptoms improved within two weeks of rituximab treatment. At the 6-month follow-up, all neurological symptoms steadily improved with steroid reduction, and both the anti-CNTN1 antibody titer and CD19+ B cells steadily decreased. No adverse events were observed after this single low-dose rituximab treatment.ConclusionsWe confirmed the clinical efficacy of low-dose rituximab by B cell depletion in autoimmune nodopathy with anti-CNTN1 antibody. This rapid and long-lasting response suggests that low-dose rituximab is a promising option for anti-CNTN1 nodopathy.

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Rituximab Responsive Relapsing–Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review

Cited by 3 publications

References 20 publications

Autoimmune nodopathy

Autoimmune nodopathy

Characteristics of anti-contactin1 antibody positive autoimmune nodopathies combined with membranous nephropathy

Effect of low-dose rituximab treatment on autoimmune nodopathy with anti-contactin 1 antibody

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