{"@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/1362544419934935680.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1136/ard.2008.089169"}},{"identifier":{"@type":"URI","@value":"https://api.elsevier.com/content/article/PII:S0003496724216873?httpAccept=text/xml"}},{"identifier":{"@type":"URI","@value":"https://api.elsevier.com/content/article/PII:S0003496724216873?httpAccept=text/plain"}},{"identifier":{"@type":"URI","@value":"https://syndication.highwire.org/content/doi/10.1136/ard.2008.089169"}},{"identifier":{"@type":"PMID","@value":"20026033"}}],"dc:title":[{"@value":"Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders"}],"description":[{"notation":[{"@value":"Mikulicz's disease (MD) has been considered as one manifestation of Sjögren's syndrome (SS). Recently, it has also been considered as an IgG(4)-related disorder.To determine the differences between IgG(4)-related disorders including MD and SS.A study was undertaken to investigate patients with MD and IgG(4)-related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG(4)-positive multiorgan lymphoproliferative syndrome (IgG(4)+MOLPS). The preliminary diagnostic criteria include raised serum levels of IgG(4) (135 mg/dl) and infiltration of IgG(4)(+) plasma cells in the tissue (IgG(4)+/IgG+ plasma cells50%) with fibrosis or sclerosis. The clinical features, laboratory data and pathologies of 64 patients with IgG(4)+MOLPS and 31 patients with typical SS were compared.The incidence of xerostomia, xerophthalmia and arthralgia, rheumatoid factor and antinuclear, antiSS-A/Ro and antiSS-B/La antibodies was significantly lower in patients with IgG(4)+MOLPS than in those with typical SS. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG(2), IgG(4) and IgE levels were significantly increased in IgG(4)+MOLPS. Histological specimens from patients with IgG(4)+MOLPS revealed marked IgG(4)+ plasma cell infiltration. Many patients with IgG(4)+MOLPS had lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG(4)+ cells were seen in the tissue of patients with typical SS. Thirty-eight patients with IgG(4)+MOLPS treated with glucocorticoids showed marked clinical improvement.Despite similarities in the involved organs, there are considerable clinical and pathological differences between IgG(4)+MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG(4)+MOLPS."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1380004053934246403","@type":"Researcher","foaf:name":[{"@value":"Y Masaki"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935695","@type":"Researcher","foaf:name":[{"@value":"L Dong"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935554","@type":"Researcher","foaf:name":[{"@value":"N Kurose"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935686","@type":"Researcher","foaf:name":[{"@value":"K Kitagawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935685","@type":"Researcher","foaf:name":[{"@value":"Y Morikawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935553","@type":"Researcher","foaf:name":[{"@value":"M Yamamoto"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935694","@type":"Researcher","foaf:name":[{"@value":"H Takahashi"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935809","@type":"Researcher","foaf:name":[{"@value":"Y Shinomura"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935813","@type":"Researcher","foaf:name":[{"@value":"K Imai"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935812","@type":"Researcher","foaf:name":[{"@value":"T Saeki"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935687","@type":"Researcher","foaf:name":[{"@value":"A Azumi"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935693","@type":"Researcher","foaf:name":[{"@value":"S Nakada"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935682","@type":"Researcher","foaf:name":[{"@value":"E Sugiyama"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935698","@type":"Researcher","foaf:name":[{"@value":"S Matsui"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935811","@type":"Researcher","foaf:name":[{"@value":"T Origuchi"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935696","@type":"Researcher","foaf:name":[{"@value":"S Nishiyama"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935702","@type":"Researcher","foaf:name":[{"@value":"I Nishimori"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935700","@type":"Researcher","foaf:name":[{"@value":"T Nojima"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935683","@type":"Researcher","foaf:name":[{"@value":"K Yamada"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935689","@type":"Researcher","foaf:name":[{"@value":"M Kawano"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935681","@type":"Researcher","foaf:name":[{"@value":"Y Zen"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935688","@type":"Researcher","foaf:name":[{"@value":"M Kaneko"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935684","@type":"Researcher","foaf:name":[{"@value":"K Miyazaki"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935810","@type":"Researcher","foaf:name":[{"@value":"K Tsubota"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935699","@type":"Researcher","foaf:name":[{"@value":"K Eguchi"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935690","@type":"Researcher","foaf:name":[{"@value":"K Tomoda"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935697","@type":"Researcher","foaf:name":[{"@value":"T Sawaki"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935701","@type":"Researcher","foaf:name":[{"@value":"T Kawanami"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935680","@type":"Researcher","foaf:name":[{"@value":"M Tanaka"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935808","@type":"Researcher","foaf:name":[{"@value":"T Fukushima"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935691","@type":"Researcher","foaf:name":[{"@value":"S Sugai"}]},{"@id":"https://cir.nii.ac.jp/crid/1382544419934935692","@type":"Researcher","foaf:name":[{"@value":"H Umehara"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"00034967"},{"@type":"PISSN","@value":"http://id.crossref.org/issn/00034967"}],"prism:publicationName":[{"@value":"Annals of the Rheumatic Diseases"}],"dc:publisher":[{"@value":"Elsevier BV"}],"prism:publicationDate":"2009-08","prism:volume":"68","prism:number":"8","prism:startingPage":"1310","prism:endingPage":"1315"},"reviewed":"false","dcterms:accessRights":"http://purl.org/coar/access_right/c_abf2","dc:rights":["https://www.elsevier.com/tdm/userlicense/1.0/","https://www.elsevier.com/legal/tdmrep-license"],"url":[{"@id":"https://api.elsevier.com/content/article/PII:S0003496724216873?httpAccept=text/xml"},{"@id":"https://api.elsevier.com/content/article/PII:S0003496724216873?httpAccept=text/plain"},{"@id":"https://syndication.highwire.org/content/doi/10.1136/ard.2008.089169"}],"createdAt":"2008-08-14","modifiedAt":"2025-01-19","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Adult","dc:title":"Adult"},{"@id":"https://cir.nii.ac.jp/all?q=Male","dc:title":"Male"},{"@id":"https://cir.nii.ac.jp/all?q=Adolescent","dc:title":"Adolescent"},{"@id":"https://cir.nii.ac.jp/all?q=Biopsy","dc:title":"Biopsy"},{"@id":"https://cir.nii.ac.jp/all?q=Prednisolone","dc:title":"Prednisolone"},{"@id":"https://cir.nii.ac.jp/all?q=Salivary%20Glands,%20Minor","dc:title":"Salivary Glands, Minor"},{"@id":"https://cir.nii.ac.jp/all?q=Mikulicz'%20Disease","dc:title":"Mikulicz' Disease"},{"@id":"https://cir.nii.ac.jp/all?q=Diagnosis,%20Differential","dc:title":"Diagnosis, Differential"},{"@id":"https://cir.nii.ac.jp/all?q=Young%20Adult","dc:title":"Young Adult"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Glucocorticoids","dc:title":"Glucocorticoids"},{"@id":"https://cir.nii.ac.jp/all?q=Aged","dc:title":"Aged"},{"@id":"https://cir.nii.ac.jp/all?q=Retrospective%20Studies","dc:title":"Retrospective Studies"},{"@id":"https://cir.nii.ac.jp/all?q=Aged,%2080%20and%20over","dc:title":"Aged, 80 and over"},{"@id":"https://cir.nii.ac.jp/all?q=Lacrimal%20Apparatus","dc:title":"Lacrimal Apparatus"},{"@id":"https://cir.nii.ac.jp/all?q=Syndrome","dc:title":"Syndrome"},{"@id":"https://cir.nii.ac.jp/all?q=Middle%20Aged","dc:title":"Middle Aged"},{"@id":"https://cir.nii.ac.jp/all?q=Magnetic%20Resonance%20Imaging","dc:title":"Magnetic Resonance Imaging"},{"@id":"https://cir.nii.ac.jp/all?q=Lymphoproliferative%20Disorders","dc:title":"Lymphoproliferative Disorders"},{"@id":"https://cir.nii.ac.jp/all?q=Sjogren's%20Syndrome","dc:title":"Sjogren's Syndrome"},{"@id":"https://cir.nii.ac.jp/all?q=Immunoglobulin%20G","dc:title":"Immunoglobulin G"},{"@id":"https://cir.nii.ac.jp/all?q=Female","dc:title":"Female"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050001335731815808","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Utility of FDG PET/CT in IgG4-related systemic disease."}]},{"@id":"https://cir.nii.ac.jp/crid/1050001335983875072","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"ja","@value":"Clinical aspects of IgG4-related orbital 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The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004236342391808","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Regulatory T Cells in Type 1 Autoimmune Pancreatitis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004240120598400","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System"}]},{"@id":"https://cir.nii.ac.jp/crid/1360013168744453632","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Comprehensive analysis of protein-expression changes specific to immunoglobulin G4-related 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Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283693982269824","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285705356903168","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Current Concept of IgG4-Related Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285706278727680","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Epidural spinal tumor and periaortitis as rare complications of Wegener’s granulomatosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285706425264768","@type":"Article","resourceType":"学術雑誌論文(journal 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Glomerulonephritis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285712600194816","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Changes in N-glycans of IgG4 and its relationship with the existence of hypocomplementemia and individual organ involvement in patients with IgG4-related disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360289848637149312","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Application of regenerative medicine to salivary gland hypofunction"}]},{"@id":"https://cir.nii.ac.jp/crid/1360298757180536320","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360302866844987776","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"New insights into chronic rhinosinusitis associated with IgG4-related disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565164390652928","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Involvement of activation of Toll‐like receptors and nucleotide‐binding oligomerization domain–like receptors in enhanced IgG4 responses in autoimmune pancreatitis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565165279903360","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Toll-like receptor activation in basophils contributes to the development of IgG4-related disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565165279933568","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Current concept and diagnosis of IgG4-related disease in the hepato-bilio-pancreatic system"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565165302333056","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Risk of malignancies in IgG4-related disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565165302344192","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565168620009856","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Prevalence of IgG4-Related 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