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Case Report

Multi-Organ Involvement of Immunoglobulin G4-Related Disease

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Gastroenterology Department, Sahloul University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse 4054, Tunisia
2
Department of Pathology, Sahloul University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse 4054, Tunisia
*
Author to whom correspondence should be addressed.
Academic Editors: Chien-Feng Li, Ching-Chieh Yang and Nai-Jung Chiang
Gastroenterol. Insights 2021, 12(3), 350-357; https://0-doi-org.brum.beds.ac.uk/10.3390/gastroent12030033
Received: 4 July 2021 / Revised: 10 August 2021 / Accepted: 10 August 2021 / Published: 21 August 2021
(This article belongs to the Special Issue Advances in Gastrointestinal Cancer)
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition of unknown etiology, with presumed autoimmune mechanisms. It is characterized by high levels of IgG4 and variable clinical manifestations. It can involve one or multiple organs. Herein, we reported the case of a 62-year-old man with three organs involvement. He initially presented with recurrent jaundice. Laboratory analysis revealed cholestasis, high gamma-globulin levels, renal failure, and proteinuria. Abdominal Magnetic Resonance Imaging (MRI) showed segmental strictures of the left intrahepatic bile ducts and the wirsung duct with an increased volume of the pancreas and diffuse bilateral enlargement of the kidneys. Laboratory tests revealed high IgG4 levels (770 mg/dL). Based on the biological and radiological findings, we have suggested the diagnosis of systemic IgG4-related disease involving bile ducts, the pancreas, and probably the kidneys. Renal biopsy revealed lymphoplasmacytic infiltrate and fibrosis, but no IgG4-positive cell. The patient received corticosteroid therapy with a complete resolution of all symptoms and a rapid normalization of all blood tests. The present case underlines the complexity of IgG4-RD because of its variable clinical presentation. The diagnosis is challenging and should be carefully assessed for possible multi-organ involvement. View Full-Text
Keywords: immunoglobulin G4-related disease; autoimmune pancreatitis; cholangitis; steroids immunoglobulin G4-related disease; autoimmune pancreatitis; cholangitis; steroids
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MDPI and ACS Style

Nour, E.; Hammami, A.; Missaoui, N.; Bdioui, A.; Dahmani, W.; Ben Ameur, W.; Braham, A.; Ajmi, S.; Ben Slama, A.; Ksiaa, M.; Jaziri, H.; Hmissa, S.; Jmaa, A. Multi-Organ Involvement of Immunoglobulin G4-Related Disease. Gastroenterol. Insights 2021, 12, 350-357. https://0-doi-org.brum.beds.ac.uk/10.3390/gastroent12030033

AMA Style

Nour E, Hammami A, Missaoui N, Bdioui A, Dahmani W, Ben Ameur W, Braham A, Ajmi S, Ben Slama A, Ksiaa M, Jaziri H, Hmissa S, Jmaa A. Multi-Organ Involvement of Immunoglobulin G4-Related Disease. Gastroenterology Insights. 2021; 12(3):350-357. https://0-doi-org.brum.beds.ac.uk/10.3390/gastroent12030033

Chicago/Turabian Style

Nour, Elleuch, Aya Hammami, Nabiha Missaoui, Ahlem Bdioui, Wafa Dahmani, Wafa Ben Ameur, Ahlem Braham, Salem Ajmi, Aida Ben Slama, Mehdi Ksiaa, Hanen Jaziri, Sihem Hmissa, and Ali Jmaa. 2021. "Multi-Organ Involvement of Immunoglobulin G4-Related Disease" Gastroenterology Insights 12, no. 3: 350-357. https://0-doi-org.brum.beds.ac.uk/10.3390/gastroent12030033

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