Open AccessNavigating the Challenges Associated With a Diagnosis of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis
Author(s) -
Osman Yılmaz,
Karen Pinto,
Vikram Deshpande
Publication year - 2022
Publication title -
archives of pathology and laboratory medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.79
H-Index - 117
eISSN - 1543-2165
pISSN - 0003-9985
DOI - 10.5858/arpa.2021-0549-ra
Subject(s) - autoimmune pancreatitis , medicine , pancreatitis , serology , pancreas , context (archaeology) , pathology , autoimmune disease , dermatology , disease , gastroenterology , immunology , antibody , paleontology , biology
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, radiographic 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 diseases, autoimmune pancreatitis, including type 1 and type 2 autoimmune pancreatitis, and immunoglobulin G4-related sclerosing cholangitis (IgG4-RSC) are particularly challenging 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.