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IgG4-related disease for the hematologist.

Hematology Am Soc Hematol Educ Program

December 2024

Division of Hematology, University of British Columbia, Vancouver, British Columbia, Canada; and Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.

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Article Synopsis
  • IgG4-related disease is an immune disorder that can cause hypertrophic spinal pachymeningitis (HSP), leading to spinal cord and nerve root compression due to infiltration of IgG4-positive plasma cells in the meninges.* -
  • A case study of a 45-year-old man with cervical myelopathy showed that surgical decompression and subsequent treatment with methylprednisolone and rituximab led to a full neurological recovery, highlighting effective management of this condition.* -
  • An updated literature review covering 52 cases revealed that neurological impairment worsened in 58% of patients, with most lesions affecting the thoracic and cervical spine, indicating the complexities and challenges in diagnosing and treating IgG4-related H
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Immunoglobulin G4-related spinal pachymeningitis: A case report.

World J Clin Cases

November 2024

Department of Physical Medicine & Rehabilitation, Jeonbuk National University Medical School, Jeonju 54907, South Korea.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a complex immune-mediated condition that causes fibrotic inflammation in several organs. A significant clinical feature of IgG4-RD is hypertrophic pachymeningitis, which manifests as inflammation of the dura mater in intracranial or spinal regions. Although IgG4-RD can affect multiple areas, the spine is a relatively rare site compared to the more frequent involvement of intracranial structures.

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A case report: Hypertrophic pachymeningitis and IgG4-related disease.

Int J Rheum Dis

November 2024

Departamento de Medicina Interna, Serviço de Reumatologia, Universidade Federal da Paraíba, João Pessoa, PB, Brazil.

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Article Synopsis
  • - The study aimed to evaluate the use of F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) in diagnosing and managing rheumatic diseases, analyzing its clinical application and imaging characteristics from 486 patient cases between 2012 and 2018.
  • - Results showed that 62.55% of the patients were female, with the majority (46.71%) aged 45-65; the primary reasons for PET/CT exams were to rule out cancers (55.56%), aid in diagnosis (24.60%), and assess disease activity (19.84%).
  • - Out of the 486 patients, 327 cases provided useful diagnostic information about rheumatic diseases,
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