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CASE REPORT article

Front. Immunol.

Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders

Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1682211

This article is part of the Research TopicCase Reports in Autoimmune and Autoinflammatory Disorders: Volume IIView all 27 articles

Rituximab for Antibody-Negative Combined Central and Peripheral Demyelination Presenting with Acute Respiratory Failure: A Case Report and Literature Review

Provisionally accepted
Yuanjiang  ZhengYuanjiang Zheng1*Jiangyan  Jiangyan HouJiangyan Jiangyan Hou2Youjun  JiangYoujun Jiang1Jianglin  Jianglin YuJianglin Jianglin Yu2Ni  Ni ZhangNi Ni Zhang1Xianwei  YeXianwei Ye1*
  • 1Guizhou Provincial People's Hospital, Guiyang, China
  • 2Zunyi Medical University, Zunyi, China

The final, formatted version of the article will be published soon.

Antibody-negative combined central and peripheral demyelination (CCPD) is a rare neuroimmunological disorder with heterogeneous manifestations and variable treatment response. We report a case of a 40-year-old man who developed acute hypercapnic respiratory failure following influenza A infection. Neuroimaging revealed demyelinating lesions in the brainstem and spinal cord, and nerve conduction studies showed multifocal peripheral demyelination. Peripheral immune profiling demonstrated marked B-cell activation and elevated serum interleukin-6 (IL-6). The patient responded poorly to corticosteroids, intravenous immunoglobulin, and plasma exchange, but improved rapidly after two consecutive daily doses of 100 mg rituximab (RTX), which was accompanied by a significant reduction in IL-6 levels, CD19⁺ B-cell depletion, ventilator weaning, and marked neurological recovery. This case underscores the importance of considering neuroimmunological causes in acute hypercapnic respiratory failure of unclear etiology. The close temporal association between IL-6 decline, B-cell depletion, and clinical improvement suggests that the B-cell–IL-6 axis may play a central role in antibody-negative CCPD pathogenesis. Low-dose RTX may represent a safe and effective therapeutic option in refractory cases.

Keywords: Combined central and peripheral demyelination, respiratory failure, B cells, IL-6, rituximab, Immunotherapy

Received: 08 Aug 2025; Accepted: 02 Oct 2025.

Copyright: © 2025 Zheng, Jiangyan Hou, Jiang, Jianglin Yu, Ni Zhang and Ye. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence:
Yuanjiang Zheng, 896476715@qq.com
Xianwei Ye, yxw1205@163.com

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