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Inicio Revista Española de Medicina Nuclear e Imagen Molecular (English Edition) IgG4-related disease
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Vol. 40. Issue 2.
Pages 107-114 (March - April 2021)
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Vol. 40. Issue 2.
Pages 107-114 (March - April 2021)
Special collaboration
IgG4-related disease
Enfermedad relacionada con IgG4
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M. Simó-Perdigóa,
Corresponding author
marc.simo@quironsalud.es

Corresponding author.
, F. Martinez-Valleb
a Servicio de Medicina Nuclear, Hospital Vall d'Hebron, Barcelona, Spain
b Servicio de Medicina Interna, Unidad de Enfermedades Sistémicas, Hospital Universitario Vall d'Hebron, Barcelona, Spain
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Table 1. Diseases within the spectrum of disease related to IgG4 and the organs affected (in parenthesis).
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:
IgG4-related disease
Autoimmune pancreatitis
18F-FDG-PET/CT
Index response
Plasmablasts
Resumen

La enfermedad relacionada con IgG4 (ER-IgG4) es una enfermedad sistémica reconocida. Se describió tras observarse que pacientes diagnosticados de pancreatitis autoinmune mostraban signos de enfermedad extra-pancreática. La clínica de estos pacientes es subaguda y se manifiesta por aparición de lesiones pseudotumorales, o tumores inflamatorios o fibrosos. En ocasiones puede ser grave como en la caso de pacientes con colangitis o vasculitis de gran vaso. Los criterios diagnósticos incluyen entre otros elevación sérica del valor de IgG4 y/o parámetros histológicos. La 18F-FDG-PET/TC es útil en el diagnóstico inicial, guía de biopsia así como en la valoración de respuesta a la terapia. Habitualmente responde al tratamiento con corticoides.

Palabras clave:
Enfermedad relacionada con IgG4
Pancreatitis autoimmune
18F-FDG-PET/TC
Responder index
Plasmablastos

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