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Neurologic Complications of Immunoglobulin G4-Related Disease (IgG4-RD)

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Neurorheumatology

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Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated systemic disease that has a particular predilection for glandular and connective tissue, including orbital adnexa and the pachymeninges. IgG4-RD enters into the differential diagnoses of numerous inflammatory, infectious, and neoplastic disorders and has emerged as a unifying diagnosis for a number of conditions once considered unrelated but now recognized to be linked by consistent histopathological features across all organs affected. The histopathological hallmarks of IgG4-RD include a lymphoplasmacytic infiltrate with a predominance of IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis. IgG4-RD generally responds well to high doses of glucocorticoids, but disease recurrences are common upon the tapering of glucocorticoids, and many patients are steroid dependent. Patients with IgG4-RD characterized by advanced fibrosis within affected organs may respond less readily to immunosuppressive therapy.

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Author information

Authors and Affiliations

  1. Department of Neurology, Harvard Medical School, Boston, MA, USA

    Bart K. Chwalisz

  2. Department of Neurology, Massachusetts General Hospital, Boston, MA, USA

    Bart K. Chwalisz

  3. Department of Rheumatology, Massachusetts General Hospital, Boston, MA, USA

    John H. Stone

Authors
  1. Bart K. Chwalisz
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  2. John H. Stone
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Correspondence to John H. Stone .

Editor information

Editors and Affiliations

  1. Division of Neuroimmunology, University of Iowa, Iowa City, IA, USA

    Tracey A. Cho

  2. Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Shamik Bhattacharyya

  3. Division of Rheumatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Simon Helfgott

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Chwalisz, B.K., Stone, J.H. (2019). Neurologic Complications of Immunoglobulin G4-Related Disease (IgG4-RD). In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_10

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  • DOI: https://doi.org/10.1007/978-3-030-16928-2_10

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-16927-5

  • Online ISBN: 978-3-030-16928-2

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