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CIDP Refractory to Three Types of Mainstay Treatments: Differential Diagnosis and Treatment Strategies Masahiro Iijima 1 1Department of Advanced Medicine, Nagoya University Graduate School of Medicine Keyword: 慢性炎症性脱髄性多発根ニューロパチー , CIDP , autoimmune nodopathy , 治療 , 進行抑制療法 , 維持療法 , バイオマーカー , chronic inflammatory demyelinating polyneuropathy , therapy , treatment to reverse disease progression , maintenance treatment , biomarker pp.517-524
Published Date 2022/5/1
DOI https://doi.org/10.11477/mf.1416202074
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Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a syndrome constructed by several clinical phenotypes that share chronic inflammatory demyelination in the peripheral nervous system. While the detailed pathogenesis is not elucidated, mainstay induction therapies such as corticosteroids, IVIg, and plasma exchange, are effective for typical CIDP. However, most conventional treatments show inadequate responses in CIDP variants. Furthermore, patients with IgG4-predominant autoantibodies (anti-NF155 Ab, anti-CNTN1 Ab, and so on) show distal-predominant disability and are recognized as refractory CIDP (autoimmune nodopathy). Combining therapeutics with induction of plasma exchange following intermittent high-dose corticosteroids could be adequate for those patients. Besides, as a novel therapeutic option, rituximab is strongly expected to be a first-line for IgG4-positive autoimmune nodopathy. Some patients show relapses before the next IVIg maintenance. We can change from intravenous immunoglobulin per three weeks to weekly subcutaneous induction. Add on corticosteroids or immunosuppressants would also be helpful to the disease stability. Recently, serum NF-L has been a candidate biomarker for secondary axonal damage in CIDP. A high-level Nf-L suggests an active phase of the disease and could indicate the requirement for therapeutic intervention.


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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