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IgG4 related disease.

M Simó-Perdigó1, F Martinez-Valle2

  • 1Servicio de Medicina Nuclear, Hospital Vall d'Hebron, Barcelona, España.

Revista Espanola De Medicina Nuclear E Imagen Molecular
|February 6, 2021
PubMed
Summary
This summary is machine-generated.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition often presenting with inflammatory tumors. Diagnosis involves elevated IgG4 levels and imaging, with steroid therapy typically effective.

Keywords:
(18)F-FDG-PET/TC18F-FDG-PET/CTAutoimmune pancreatitisEnfermedad relacionada con IgG4IgG4-related diseaseIndex responsePancreatitis autoinmunePlasmablastosPlasmablastsResponder index

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Area of Science:

  • Immunology
  • Radiology
  • Pathology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition.
  • It is characterized by the development of pseudotumoral lesions and can affect multiple organs.
  • Initial descriptions arose from patients with autoimmune pancreatitis who subsequently developed extra-pancreatic manifestations.

Purpose of the Study:

  • To summarize the key aspects of IgG4-related disease.
  • To highlight diagnostic criteria and imaging utility.
  • To discuss treatment response.

Main Methods:

  • Review of clinical manifestations and diagnostic parameters of IgG4-RD.
  • Emphasis on the role of 18F-FDG-PET/CT in diagnosis and monitoring.
  • Discussion of therapeutic approaches.

Main Results:

  • IgG4-RD presents with subacute clinical manifestations, including pseudotumoral lesions.
  • Serious complications can arise, such as cholangitis and large vessel vasculitis.
  • Diagnostic criteria involve serum IgG4 elevation and/or histological findings.

Conclusions:

  • 18F-FDG-PET/CT is valuable for initial diagnosis, guiding biopsies, and assessing treatment response in IgG4-RD.
  • The disease typically shows a positive response to steroid therapy.