IgG4-Related Sclerosing Cholangitis in America
Since its first description in the 1960s from the United States, IgG4-SC has been increasingly recognized and reported from around the world. It is the most common extrapancreatic manifestation of type 1 autoimmune pancreatitis and is possibly associated with an accelerated development of secondary biliary cirrhosis if diagnosis is delayed. Obstructive jaundice and constitutional symptoms are the main presenting symptoms, similar to hilar cholangiocarcinoma and primary sclerosing cholangitis, which are the most important differential diagnoses of IgG4-SC. Endoscopy is critical to rule out malignancy. While steroids are the mainstay of initial treatment, and most patients respond rapidly, there is a very high likelihood of relapse, especially in patients with proximal biliary strictures. Initiation of immunomodulators or rituximab may be required to maintain long-term remission. In this chapter we will describe the epidemiology, diagnosis, clinical presentation, and management of IgG4-SC based on studies primarily from the United States.
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