Document Detail

Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders.
MedLine Citation:
PMID:  18701557     Owner:  NLM     Status:  MEDLINE    
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.
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:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-08-13
Journal Detail:
Title:  Annals of the rheumatic diseases     Volume:  68     ISSN:  1468-2060     ISO Abbreviation:  Ann. Rheum. Dis.     Publication Date:  2009 Aug 
Date Detail:
Created Date:  2009-07-16     Completed Date:  2009-09-03     Revised Date:  2010-02-19    
Medline Journal Info:
Nlm Unique ID:  0372355     Medline TA:  Ann Rheum Dis     Country:  England    
Other Details:
Languages:  eng     Pagination:  1310-5     Citation Subset:  IM    
Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa, 920-0293, Japan.
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MeSH Terms
Aged, 80 and over
Diagnosis, Differential
Glucocorticoids / therapeutic use
Immunoglobulin G / analysis*
Lacrimal Apparatus / pathology
Lymphoproliferative Disorders / diagnosis,  drug therapy,  immunology*,  pathology
Magnetic Resonance Imaging
Middle Aged
Mikulicz' Disease / diagnosis,  drug therapy,  immunology*,  pathology
Prednisolone / therapeutic use
Retrospective Studies
Salivary Glands, Minor / pathology
Sjogren's Syndrome / diagnosis,  immunology,  pathology
Young Adult
Reg. No./Substance:
0/Glucocorticoids; 0/Immunoglobulin G; 50-24-8/Prednisolone
Comment In:
Gastroenterology. 2010 Feb;138(2):781-4   [PMID:  20026033 ]

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