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Review of IgG4-related disease
Revisión de la enfermedad relacionada con la IgG4
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Raquel Sánchez-Oroa,b,
Corresponding author
raquel_sanchez_oro@hotmail.com

Corresponding author.
, Elsa María Alonso-Muñoza, Lidia Martí Romeroc
a Servicio de Radiodiagnóstico, Hospital Arnau de Vilanova, Valencia, Spain
b Servicio de Radiodiagnóstico, Hospital Obispo Polanco, Teruel, Spain
c Servicio de Medicina Digestiva, Hospital Francesc de Borja, Gandía, Valencia, Spain
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Received 21 March 2019. Accepted 18 August 2019
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Abstract

IgG4-related disease is a fibrous-inflammatory process related to immunomodulation. The most commonly affected organs are: the pancreas, bile duct, major salivary glands, lacrimal glands, retroperitoneum and lymphatic ducts.

In recent decades, this disease has been recognised as a systemic disorder that includes many single organ disorders, previously unrelated and known as independent entities.

The common characteristics shared by the different entities that make up the IgG4-related disease are: raised serum IgG4 levels, alterations in the imaging tests with neoplastic-like swelling of the affected organs, specific histopathological characteristics and in immunostaining, as well as good response to treatment with glucocorticoids.

In this work, we will review this pathology with a special emphasis on the characteristics of autoimmune pancreatitis, sclerosing cholangitis related to IgG4 and the involvement of the retroperitoneum, mesenterium and the digestive tract.

Keywords:
IgG4-related disease
Autoimmune pancreatitis
Resumen

La enfermedad relacionada con la IgG4 es un proceso fibroinflamatorio relacionado con la inmumomediación. Los órganos que se ven afectados por esta enfermedad más frecuentemente son: el páncreas, la vía biliar, las glándulas salivares mayores, lacrimales, retroperitoneo y linfáticos.

En las últimas décadas esta enfermedad ha sido reconocida como un trastorno sistémico que engloba a muchas afecciones individuales de órganos, antes no relacionadas y conocidas como entidades independientes.

Las características comunes compartidas por las distintas entidades que componen la ER-IgG4 son: cifras elevadas de IgG4 sérica, alteraciones en las pruebas de imagen con tumefacción de aspecto neoplásico de los órganos afectados, características histopatológicas propias y en inmunotinción, buena respuesta al tratamiento con glucocorticoides.

En este trabajo realizaremos una revisión de esta patología con especial énfasis en las características de la pancreatitis autoinmune, colangitis esclerosante relacionada con IgG4, el compromiso del retroperitoneo y mesenterio, y del tubo digestivo.

Palabras clave:
Enfermedad relacionada con IgG4
Pancreatitis autoinmune

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