Korean J Gastroenterol.  2020 May;75(5):257-263. 10.4166/kjg.2020.75.5.257.

Recent Updates of Immunoglobulin G4-related Pancreatobiliary Disease

  • 1Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea


Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis (IgG4-SC) are the pancreatobiliary manifestations of IgG4-related disease. IgG4-related disease is a newly named fibroinflammatory condition that is characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells and often by elevated serum IgG4 concentrations. IgG4-related pancreatobiliary disease is often disguised as pancreatobiliary malignancies owing to its tumefactive nature and clinical presentations, such as obstructive jaundice. The differentiation of IgG4-SC from primary sclerosing cholangitis is also essential because of the significant differences in treatment responses and prognosis. A timely diagnosis of IgG4-related pancreatobiliary disease can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse the pancreatobiliary duct strictures and obstructive jaundice. On the other hand, the diagnosis of IgG4-related pancreatobiliary disease is sometimes challenging because there is no single diagnostic clinical test. The diagnosis of IgG4-related pancreatobiliary disease rests on fulfilling the diagnostic criteria, including imaging, serology, other organ involvement, histology, and response to steroids. Approximately 50% of patients with IgG4-related pancreatobiliary disease experience relapse, despite IgG4-related pancreatobiliary disease showings a favorable short-term prognosis after glucocorticoid therapy. To reduce the relapse, long maintenance treatment for 3 years may be necessary. The purposes of this review were to emphasize the clinical problem of diagnosing IgG4-related pancreatobiliary disease as well as to highlight the use of the published guidelines for the diagnosis and management of IgG4-related pancreatobiliary disease.


Autoimmune pancreatitis; IgG4-related sclerosing cholangitis; Immunoglobulin G4-related disease
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