Abstract
Autoimmune pancreatitis (AIP) was reported in 2001 to be associated with high serum immunoglobulin (Ig)G4 levels [1]. Thereafter, elevated serum IgG4 was also detected in other diseases, such as dacryoadenitis/sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, lung disease, and sclerosing cholangitis (SC), which led to the proposal of a new disease concept called IgG4-related disease (IgG4-RD). Roughly 60–90% of patients with IgG4-related SC (IgG4-SC) have concomitant AIP and are relatively easy to identify. However, those with no increase in serum IgG4 and/or biliary lesions only can be difficult to diagnose. In particular, differentiation from hilar cholangiocarcinoma and primary SC (PSC) is challenging in IgG4-SC patients with biliary stenosis, which encompasses the hepatic portal and intrahepatic regions. While imaging and histopathological findings remain the most important means of diagnosing each disease, increased serum IgG4 levels have also become a useful diagnostic aid. In this chapter, we describe the roles and properties of serum IgG4, the relevance of serum IgG4 in various diseases, and finally outline the diagnostic value of serum IgG4 for IgG4-SC.
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Ito, T., Watanabe, T., Muraki, T., Kawa, S. (2019). Serum IgG4. In: Kamisawa, T., Kim, MH. (eds) IgG4-Related Sclerosing Cholangitis. Springer, Singapore. https://doi.org/10.1007/978-981-10-4548-6_6
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DOI: https://doi.org/10.1007/978-981-10-4548-6_6
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