AN OVERLAPPING CASE OF IGG4-RELATED DISEASE AND SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a rare newly discovered systemic immune-mediated fibroinflammatory disease of unknown etiology that can affect multiple organs. Involvement of the pancreas and biliary tract is the most common and well described in the literature. It shares certain similarities with cholangiocarcinoma. IgG4 sclerosing cholangitis (IgG4-SC) is an immune-mediated process that results in inflammation and fibrosis of the pancreatobiliarytract. Although IgG4-SC is predominantly associated with autoimmune pancreatitis, IgG4-SC as its own entity can be difficult to diagnose. Our case is presenting the fact that systemic lupus erythematosus and autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis, revealed by jaundice with cholestasis disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging. There are still significant gaps in our understanding, particularly in terms of pathogenesis and factors that influence therapy response. Further observational and interventional research is needed to better manage this disease.