IgG4-Related Disease Presenting as Hypertrophic Pachymeningitis

IgG4-related disease (IgG4-RD) is a multi-organ, immune-mediated inflammatory condition of unknown etiology characterized by infiltration of tissues by IgG4 producing plasma cells. IgG4-related disease (IgG4-RD) can ideally affect any organs, but the involvement of the central nervous system (CNS) i...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2022-02, Vol.14 (2), p.e21850
Main Authors: Sapkota, Binita, Rampure, Ritesh, Gokden, Murat, Kanuru, Sruthi
Format: Article
Language:English
Subjects:
Antigens
Biopsy
Case reports
Diplopia
Hepatitis
Inflammatory diseases
Internal Medicine
Magnetic resonance imaging
Plasma
Proteins
Rheumatoid arthritis
Rheumatology
Steroids
Ulcers
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Summary:IgG4-related disease (IgG4-RD) is a multi-organ, immune-mediated inflammatory condition of unknown etiology characterized by infiltration of tissues by IgG4 producing plasma cells. IgG4-related disease (IgG4-RD) can ideally affect any organs, but the involvement of the central nervous system (CNS) is a rare entity. We present a case of a 67-year-old male who presented with diplopia with imaging showing hypertrophic pachymeningitis (HPM) and was diagnosed with IgG4-RD of the CNS based on histopathology report.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.21850