Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Authors

DOI:

https://doi.org/10.22516/25007440.978

Keywords:

IgG4-Related Disease, autoimmune pancreatitis, Immunoglobulin G4

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner’s tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

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Author Biographies

Henry Tomas Vargas Marcacuzco, Hospital Nacional Guillermo Almenara Irigoyen

Médico gastroenterólogo asistente en el serivico de gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen. Lima, Perú.

Sonia Irene Junes Pérez, Hospital Nacional Guillermo Almenara Irigoyen

Médico gastroenterólogo, asistente en el servicio de gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen. Lima Perú.

Rocio Elizabeth Santos Julián , Hospital Nacional Guillermo Almenara Irigoyen

Médica asistente en el servicio de radiología, Hospital Nacional Guillermo Almenara Irigoyen. Lima Perú.

Ricardo Alejandro Rocha Bolaños, Hospital Nacional Guillermo Almenara Irigoyen

Médico residente en el servicio de gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen. Lima, Perú.

Jessica Matheus Sairitupac, Hospital Nacional Guillermo Almenara Irigoyen

Médico asistente en el servicio de anatomía patológica del Hospital Nacional Guillermo Almenara Irigoyen. Lima, Perú.

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Figura 1. A. La tomografía axial computarizada (TAC) sin contraste 2 años antes de la admisión muestra un aumento de volumen de la glándula submandibular izquierda, asociado con algunas adenopatías adyacentes. B. La TAC con contraste muestra un halo hipoatenuante característico alrededor del cuerpo y la cola del páncreas, un hallazgo sugestivo de pancreatitis autoinmune difusa. C y D. La reconstrucción MinIP y la colangiorresonancia muestran una dilatación de la vía biliar intrahepática (asterisco), así como áreas de estenosis segmentarias (estrella). E. Imagen de resonancia magnética axial ponderada en T2 que muestra un agrandamiento focal bien delimitado a nivel de la cabeza, que se correlaciona con restricción en la difusión (G). F, G y H. Imagen de resonancia magnética axial ponderada en T1 con contraste que muestra múltiples lesiones nodulares renales bien definidas, las cuales muestran realce a la administración de contraste y se correlacionan con restricción a la difusión (G y H). Fuente: expediente médico del paciente

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Published

2023-12-19

How to Cite

Vargas Marcacuzco, H. T., Junes Pérez, S. I., Santos Julián , R. E., Rocha Bolaños, R. A., & Matheus Sairitupac, J. (2023). Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case. Revista Colombiana De Gastroenterología, 38(4), 521–528. https://doi.org/10.22516/25007440.978

Issue

Vol. 38 No. 4 (2023): Octubre - Diciembre

Section

Case report

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