Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment
書誌事項
- 公開日
- 2019-07-30
- 資源種別
- journal article
- 権利情報
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- http://www.springer.com/tdm
- http://www.springer.com/tdm
- DOI
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- 10.1007/s10620-019-05746-9
- 公開者
- Springer Science and Business Media LLC
この論文をさがす
説明
Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic manifestation of IgG4-RD, and type 2 characterized by granulocytic epithelial lesions. The characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmocytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extrapancreatic manifestations of IgG4-RD (e.g., sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. These entities can be differentiated from mimickers by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line therapy is corticosteroids, or rituximab in high-risk patients with steroid intolerance. Although relapse rates are high, treatment of relapsed disease remains experimental.
収録刊行物
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- Digestive Diseases and Sciences
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Digestive Diseases and Sciences 64 (9), 2385-2394, 2019-07-30
Springer Science and Business Media LLC
