{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1361412890842728448.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1007/s10620-019-05746-9"}},{"identifier":{"@type":"URI","@value":"http://link.springer.com/content/pdf/10.1007/s10620-019-05746-9.pdf"}},{"identifier":{"@type":"URI","@value":"http://link.springer.com/article/10.1007/s10620-019-05746-9/fulltext.html"}},{"identifier":{"@type":"PMID","@value":"31363956"}}],"resourceType":"学術雑誌論文(journal article)","dc:title":[{"@value":"Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment"}],"description":[{"notation":[{"@value":"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."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1420564276176860544","@type":"Researcher","personIdentifier":[{"@type":"KAKEN_RESEARCHERS","@value":"70145126"},{"@type":"NRID","@value":"1000070145126"},{"@type":"NRID","@value":"9000399220205"},{"@type":"NRID","@value":"9000002898011"},{"@type":"NRID","@value":"9000375885004"},{"@type":"NRID","@value":"9000404101484"},{"@type":"NRID","@value":"9000370746860"}],"foaf:name":[{"@value":"Kazuichi Okazaki"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"01632116"},{"@type":"EISSN","@value":"15732568"}],"prism:publicationName":[{"@value":"Digestive Diseases and Sciences"}],"dc:publisher":[{"@value":"Springer Science and Business Media LLC"}],"prism:publicationDate":"2019-07-30","prism:volume":"64","prism:number":"9","prism:startingPage":"2385","prism:endingPage":"2394"},"reviewed":"false","dc:rights":["http://www.springer.com/tdm","http://www.springer.com/tdm"],"url":[{"@id":"http://link.springer.com/content/pdf/10.1007/s10620-019-05746-9.pdf"},{"@id":"http://link.springer.com/article/10.1007/s10620-019-05746-9/fulltext.html"}],"createdAt":"2019-07-30","modifiedAt":"2020-07-28","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Autoimmune%20Pancreatitis","dc:title":"Autoimmune Pancreatitis"},{"@id":"https://cir.nii.ac.jp/all?q=Immunoglobulin%20G","dc:title":"Immunoglobulin G"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Immunoglobulin%20G4-Related%20Disease","dc:title":"Immunoglobulin G4-Related Disease"},{"@id":"https://cir.nii.ac.jp/all?q=Induction%20Chemotherapy","dc:title":"Induction Chemotherapy"},{"@id":"https://cir.nii.ac.jp/all?q=History,%2020th%20Century","dc:title":"History, 20th Century"},{"@id":"https://cir.nii.ac.jp/all?q=History,%2021st%20Century","dc:title":"History, 21st Century"},{"@id":"https://cir.nii.ac.jp/all?q=Maintenance%20Chemotherapy","dc:title":"Maintenance Chemotherapy"}],"project":[{"@id":"https://cir.nii.ac.jp/crid/1040282256945119872","@type":"Project","projectIdentifier":[{"@type":"KAKEN","@value":"17K09468"},{"@type":"JGN","@value":"JP17K09468"},{"@type":"URI","@value":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-17K09468/"}],"notation":[{"@language":"ja","@value":"自己免疫膵炎の免疫病態と線維化における自然免疫の関与に関する研究"},{"@language":"en","@value":"Role of innate immunity in the development of autoimmune pancreatisitis"}]}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050001202635028992","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: a multicentre study"}]},{"@id":"https://cir.nii.ac.jp/crid/1050001338927148800","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Type 1 autoimmune pancreatitis can transform into chronic pancreatitis: a long-term follow-up study of 73 Japanese patients"}]},{"@id":"https://cir.nii.ac.jp/crid/1360001114196222976","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 III. 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