Autoimmune pancreatitis (AIP) is a rare disease clinically characterized by obstructive jaundice, unintentional weight loss, acute pancreatitis, focal pancreatic mass, and diabetes. AIP is classified into two subtypes - type 1 and type 2 - according to pathological findings, clinical features, and serology test results, but some cases may be defined as type not otherwise in the absence of pathological findings and inflammatory bowel disease. To address the differences in diagnostic criteria by country, standard diagnostic criteria for AIP were proposed in 2011 by an international consensus of expert opinions. Differential diagnosis of AIP from pancreatic ductal adenocarcinoma is important but remains challenging for clinicians. Fortunately, all subtypes of AIP show dramatic response to steroid treatment. This review discusses the current perspectives on the diagnosis and management of AIP in clinical practice.

The emergence of glucocorticoid-responsive autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC), a new disease entity, has attracted considerable interest within the international gastroenterology community. The typical manifestations of AIP/IgG4-SC are obstructive jaundice and pancreatic enlargement in the elderly, which may mimic the presentations of pancreatobiliary malignancies. The timely diagnosis of AIP/IgG4-SC can lead to adequate glucocorticoid treatment, whereas a misdiagnosis can result in unnecessary major surgery. The diagnostic criteria used to diagnose AIP include several cardinal features of AIP that can be detected via pancreatic parenchymal imaging, ductal imaging, serum IgG4 levels, histopathology, other organ involvement, and response to glucocorticoid therapy. The differential diagnosis of AIP/IgG4-SC may include pancreatobiliary malignancies and primary sclerosing cholangitis. Although most patients with AIP/IgG4-SC respond well to glucocorticoid therapy, there is a frequent relapse of the disease in the long term. This review describes the evolution of the concept of AIP and IgG4-related disease, including the development of diagnostic criteria, discusses the current practice for diagnosis and treatment, and suggests prospects for research.

Cholestatic liver diseases (CLDs) occur as a result of bile duct injury, emanating into duct obstruction and bile stasis. Advances in radiological imaging in the last decade has replaced endoscopic retrograde cholangiopancreatography (ERCP) as the first diagnostic tool, except in certain groups of patients, such as those with ischemic cholangiopathy (IsC) or early stages of primary sclerosing cholangitis (PSC). ERCP provides an opportunity for targeted tissue acquisition for histopathological evaluation and carries a diverse therapeutic profile to restore bile flow. The aim of this review article is to appraise the diagnostic and therapeutic roles of ERCP in CLDs.

Endoscopic retrograde cholangiopancreatography is used to evaluate the narrowing of the main pancreatic duct in autoimmune pancreatitis (AIP) and biliary stricture in IgG4-related sclerosing cholangitis (IgG4-SC). Intraductal ultrasonography enables detailed visualization of the thickening of the bile duct wall in IgG4-SC. Pancreatic cancer, cholangiocarcinoma, and primary sclerosing cholangitis are important mimicking conditions of AIP and IgG4-SC. Diffuse or segmental stricture without marked upstream dilatation is a typical pancreatographic finding in AIP. By contrast, a single, short stricture with marked upstream dilatation is a typical finding in pancreatic cancer. The cholangiogram of IgG4-SC is classified into four types based on biliary stricture location, and this cholangiogram classification is useful for the differential diagnosis of IgG4-SC. Endoscopic retrograde cholangiography can be used to distinguish between IgG4-SC and primary sclerosing cholangitis. A segmental/long and intrapancreatic stricture is a characteristic finding of IgG4-SC, whereas band-like strictures, a beaded or pruned-tree appearance, and diverticulum-like outpouching are characteristic of primary sclerosing cholangitis. The characteristic intraductal ultrasonographic findings of circular-symmetrical wall thickening, smooth outer and inner margins, and homogeneous internal echo at the biliary stricture site are useful for diagnosis of IgG4-SC. Thickening of the bile duct wall at non-stricture sites is also a typical intraductal ultrasonographic finding of IgG4-SC and can be used for differential diagnosis from cholangiocarcinoma. Transpapillary bile duct and duodenal papilla biopsy during endoscopic retrograde cholangiopancreatography are also useful in the diagnosis of IgG4-SC.