| Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. | |
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MedLine Citation:
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PMID: 18701557 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Mikulicz's disease (MD) has been considered as one manifestation of Sj?gren's syndrome (SS). Recently, it has also been considered as an IgG(4)-related disorder. OBJECTIVE: To determine the differences between IgG(4)-related disorders including MD and SS. METHODS: 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 cells >50%) 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. RESULTS: 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. CONCLUSION: 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. |
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Authors:
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Y Masaki; L Dong; N Kurose; K Kitagawa; Y Morikawa; M Yamamoto; H Takahashi; Y Shinomura; K Imai; T Saeki; A Azumi; S Nakada; E Sugiyama; S Matsui; T Origuchi; S Nishiyama; I Nishimori; T Nojima; K Yamada; M Kawano; Y Zen; M Kaneko; K Miyazaki; K Tsubota; K Eguchi; K Tomoda; T Sawaki; T Kawanami; M Tanaka; T Fukushima; S Sugai; H Umehara |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2008-08-13 |
Journal Detail:
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Title: Annals of the rheumatic diseases Volume: 68 ISSN: 1468-2060 ISO Abbreviation: Ann. Rheum. Dis. Publication Date: 2009 Aug |
Date Detail:
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Created Date: 2009-07-16 Completed Date: 2009-09-03 Revised Date: 2010-02-19 |
Medline Journal Info:
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Nlm Unique ID: 0372355 Medline TA: Ann Rheum Dis Country: England |
Other Details:
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Languages: eng Pagination: 1310-5 Citation Subset: IM |
Affiliation:
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Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa, 920-0293, Japan. yasum@kanazawa-med.ac.jp |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Aged, 80 and over Biopsy Diagnosis, Differential Female Glucocorticoids / therapeutic use Humans Immunoglobulin G / analysis* Lacrimal Apparatus / pathology Lymphoproliferative Disorders / diagnosis, drug therapy, immunology*, pathology Magnetic Resonance Imaging Male Middle Aged Mikulicz' Disease / diagnosis, drug therapy, immunology*, pathology Prednisolone / therapeutic use Retrospective Studies Salivary Glands, Minor / pathology Sjogren's Syndrome / diagnosis, immunology, pathology Syndrome Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Glucocorticoids; 0/Immunoglobulin G; 50-24-8/Prednisolone |
| Comments/Corrections | |
Comment In:
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Gastroenterology. 2010 Feb;138(2):781-4
[PMID:
20026033
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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