Multi-Organ Involvement of Immunoglobulin G4-Related Disease

• Main Authors: Elleuch Nour, Aya Hammami, Nabiha Missaoui, Ahlem Bdioui, Wafa Dahmani, Wafa Ben Ameur, Ahlem Braham, Salem Ajmi, Aida Ben Slama, Mehdi Ksiaa, Hanen Jaziri, Sihem Hmissa, Ali Jmaa
• Format: Article
• Language: English
• Published: MDPI AG 2021-08-01
• Series: Gastroenterology Insights
• Subjects: immunoglobulin G4-related disease; autoimmune pancreatitis; cholangitis; steroids
• Online Access: https://www.mdpi.com/2036-7422/12/3/33

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.