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Review
IgG4-related disease in the nervous system
  1. Lu Yang1,2,
  2. R J Paul Smith3,
  3. Neil J Scolding2,4,
  4. Claire M Rice1,2
  1. 1 Department of Neurology, Southmead Hospital, North Bristol NHS Trust, Bristol, UK
  2. 2 Clinical Neurosciences, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK
  3. 3 Department of Neuroradiology, Southmead Hospital, North Bristol NHS Trust, Bristol, UK
  4. 4 Department of Neurology, Gloucester Royal Hospital, Gloucester, UK
  1. Correspondence to Dr Claire M Rice; C.M.Rice{at}bristol.ac.uk

Abstract

IgG4-related disease (IgG4-RD) is a recently described multisystemic disorder with a spectrum of manifestations that continue to be described. Nonetheless, there are recognised distinct patterns of disease. Neurological involvement is rare, particularly in isolation, but IgG4-RD may present with orbital disease, hypophysitis or pachymeningitis. Typically, it is highly responsive to treatment. This review highlights neurological manifestations of IgG4-RD and emphasises the importance of a high index of clinical suspicion to facilitate investigation and appropriate management, avoiding irreversible tissue damage and neurological dysfunction. We present a treatment algorithm for suggested management of IgG4-RD affecting the nervous system.

  • NEUROIMMUNOLOGY
  • NEUROPATHOLOGY
  • NEURORADIOLOGY
  • PATHOLOGY

Data availability statement

No data are available.

https://doi.org/10.1136/pn-2020-002863

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    Data availability statement

    No data are available.

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    Footnotes

    • Contributors LY: initial draft of the manuscript and subsequent revisions, figure creation. RJPS: specialist neuroradiology input. NJS: review of manuscript. CMR: concept for manuscript, review of manuscript and figure creation, guarantor.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed by Ruth Dobson, London, UK.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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