期刊
ANNALS OF THE RHEUMATIC DISEASES
卷 68, 期 8, 页码 1310-1315出版社
BMJ PUBLISHING GROUP
DOI: 10.1136/ard.2008.089169
关键词
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类别
资金
- Japanese Ministry of Education, Culture, Sports, Science and Technology [13557160, 15024236, 15390313, 13877075, 17591060]
- Uehara Memorial Foundation
- Kanazawa Medical University Research Foundation [C2006-1, S2004-16, S2007-5]
- Grants-in-Aid for Scientific Research [15024236, 13877075, 17591060, 13557160, 15390313] Funding Source: KAKEN
Background: Mikulicz's disease (MD) has been considered as one manifestation of Sjogren'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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