Abstract
Background: IgG4-related disease (IgG4-RD) is a progressive, chronic, atypical presenting clinical entity characterized by an elevated serum IgG4 level, leading to an immune-mediated fibroinflammatory condition affecting various organs.Case Summary: We present the case of a patient with clinical manifestations compatible with chronic pancreatitis due to the time of evolution. Physical examination revealed submaxillary, axillary, and inguinal lymphadenopathy and pain on abdominal palpation. Laboratory tests showed eosinophilia and a hepatocellular pattern in the liver profile. Immunofixation showed a monoclonal IgG lambda band with IgG4 values of 1183 mg/dl. To confirm the diagnosis of autoimmune chronic pancreatitis associated with IgG4-related sclerosing cholangitis, imaging tests were performed, which showed intrahepatic and extrahepatic cholestasis associated with pancreatic parenchymal alterations compatible with this entity. Subsequently, upon initiating glucocorticoid therapy, the patient evolved favorably. Conclusion: ER-IgG4 is a rare condition that causes inflammation and scarring in various organs. It is hard to diagnose and often mistaken for other diseases. The pancreas and biliary tract are frequently affected.
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