IgG4 related disease

M Simó-Perdigó; F Martinez-Valle

doi:10.1016/j.remn.2020.12.001

IgG4 related disease

Rev Esp Med Nucl Imagen Mol (Engl Ed). 2021 Mar-Apr;40(2):107-114. doi: 10.1016/j.remn.2020.12.001. Epub 2021 Feb 3.

[Article in English, Spanish]

Authors

M Simó-Perdigó¹, F Martinez-Valle²

Affiliations

¹ Servicio de Medicina Nuclear, Hospital Vall d'Hebron, Barcelona, España. Electronic address: marc.simo@quironsalud.es.
² Servicio de Medicina Interna, Unidad de Enfermedades Sistémicas, Hospital Universitario Vall d'Hebron, Barcelona, España.

PMID: 33547020
DOI: 10.1016/j.remn.2020.12.001

Abstract

IgG4-related disease (ER-IgG4) is a recognised systemic disease. It was described after patients diagnosed with autoimmune pancreatitis showed signs of extra-pancreatic disease. The clinical manifestation of these patients is subacute and is manifested by the appearance of pseudotumoral lesions, or inflammatory or fibrous tumours. Sometimes it can be serious as in the case of patients with cholangitis or large vessel vasculitis. Diagnostic criteria include, among others, serum IgG4 elevation and/or histological parameters. The 18F-FDG-PET/CT is useful in the initial diagnosis, biopsy guidance as well as in the assessment of response to therapy. It usually responds to steroid therapy.

Keywords: (18)F-FDG-PET/TC; 18F-FDG-PET/CT; Autoimmune pancreatitis; Enfermedad relacionada con IgG4; IgG4-related disease; Index response; Pancreatitis autoinmune; Plasmablastos; Plasmablasts; Responder index.