Abstract
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.
Highlights
IgG4-related disease (IgG4-RD) of the central nervous system (CNS) is rare, and only about 82 cases have been described in literature who have hypertrophic pachymeningitis (HPM) as a manifestation of IgG4-RD [1]
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
Serum IgG4 level can be normal in patients with meningeal IgG4-related HPM, and cerebrospinal fluid (CSF) IgG can be elevated, a meningeal biopsy is necessary to confirm the diagnosis and rule out other inflammatory, infectious, and neoplastic etiologies of hypertrophic pachymeningitis [3]
Summary
IgG4-related disease (IgG4-RD) of the central nervous system (CNS) is rare, and only about 82 cases have been described in literature who have hypertrophic pachymeningitis (HPM) as a manifestation of IgG4-RD [1]. Seven years prior to this presentation, he had whole left upper extremity numbness that lasted for 24 hours and resolved spontaneously He denied any history of inflammatory eye disease, thrombosis, or risks for tuberculosis. He denied any joint pain, joint swelling, fever, recent change in weight, skin rash, lymphadenopathy, oral ulcer, nasal ulcer, chest pain, shortness of breath, cough, hemoptysis, numbness, tingling, and muscle weakness His father had rheumatoid arthritis, and no family history of other autoimmune diseases or malignancy was reported. GMS and Ziehl-Neelsen stains were negative for fungal and acid-fast microorganisms (Figure 2) He was started on prednisone 60 mg orally daily and had resolution of diplopia. The plan is to start rituximab for steroid-sparing effect and to prevent disease relapse
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