Terumi Kamisawa - IgG4-Related Sclerosing Cholangitis

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IgG4-related disease (IgG4-RD) is a fibro-inflammatory and immune-mediated condition with a tendency toward the tumefaction that mimics malignant or inflammatory disorders. IgG4-RD was first proposed as a systemic disease in 2003 following the recognition that a high percentage of patients with autoimmune pancreatitis (AIP) had extrapancreatic lesions that shared similar histopathological findings consisting of abundant infiltration of IgG4-positive plasma cells and lymphocytes and fibrosis. IgG4-RD can affect almost any organ; only a single organ is clinically involved in some cases, whereas others show effects on two or more organs simultaneously or metachronously. IgG4-RD is also characterized by elevation of serum IgG4 levels and steroid responsiveness.

IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-RD. Although about 60% of patients with IgG4-RD have biliary lesions in the proximal and/or distal bile ducts, the inclusion of a distal biliary stricture isolated to the intrapancreatic portion associated with AIP in the definition of IgG4-SC remains under debate.

When stenosis develops in the hilar or intrahepatic bile duct, the cholangiographic appearance is similar to that of primary sclerosing cholangitis (PSC), a progressive disease for which liver transplantation is the only effective curative treatment. Another important disease that should be differentiated from IgG4-SC is cholangiocarcinoma. The radiological findings of IgG4-SC involving the hilar bile duct are quite similar to those of hilar cholangiocarcinoma. Since IgG4-SC responds well to steroid therapy, it is necessary to differentiate between the two diseases in order to provide the most appropriate treatment regimen. Although clinical diagnostic criteria of IgG4-SC were proposed in 2012, its diagnosis is still a clinical challenge and requires a multidisciplinary approach, in which serology, imaging, and histology play crucial roles.

Currently, systemic glucocorticoid is the first-line agent for IgG4-SC; however, frequent relapse of the disease remains a problem. Recently, rituximab has been successfully used to treat patients with IgG4-SC who showed resistance to or side effects from steroid treatment.

The goal of this book is to raise awareness of IgG4-SC and provide practicing physicians the principles for its diagnosis and management by clarifying its current concept and covering all aspects of its clinical, serological, histopathological, imaging, therapeutic, and prognostic features in the world. We are deeply grateful to all the authors for their painstaking writing and contributions in preparing this concise and informative book. The publisher has also made a significant contribution to this book and has turned out an impressive volume with illustrations of the highest quality.