Navigating the Challenges Associated With a Diagnosis of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis

Context.-The pancreatobiliary tract exhibits a spectrum of heterogeneous fibroinflammatory conditions that may be the result of a primary immune-mediated mechanism, or a reaction to neoplasm. This often results in significant overlap regarding clinical presentation, symptoms, radio-graphic findings, serology, and histopathology between inflammatory and neoplastic lesions of the pancreas, which can lead to inadvertent surgical intervention. Among the multitude of primary fibroinflammatory pancreatic diseas-es, autoimmune pancreatitis, including type 1 and type autoimmune pancreatitis, and immunoglobulin G4-related sclerosing cholangitis (IgG4-RSC) are particularly chal-lenging and require a multidisciplinary perspective to reliably make a diagnosis. This is of particular significance because these diseases typically have a favorable prognosis and readily respond to steroid therapy. Objective.-To present a multimodal approach to highlight distinctive and overlapping qualities that will aid in the diagnosis of these entities. Data Sources.-The review and analysis of literature describing autoimmune pancreatitis types 1 and 2 and IgG4-RSC. Conclusions.-Diagnosis of autoimmune pancreatitis types 1 and 2 and IgG4-RSC requires a multimodal approach that relies on clinical, radiographic, serologic, histopathologic, and immunohistochemical correlation.

Automated summary

The pancreatobiliary tract has heterogeneous fibroinflammatory conditions that may be immune-mediated or a reaction to neoplasm, leading to overlap between inflammatory and neoplastic lesions. Autoimmune pancreatitis types 1 and 2 and immunoglobulin G4-related sclerosing cholangitis require a multimodal approach for diagnosis, relying on clinical, radiographic, serologic, histopathologic, and immunohistochemical correlation.