Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: Analysis of 64 cases of IgG4-related disorders

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. KawanoY. Zen, M. Kaneko, K. Miyazaki, K. Tsubota, K. Eguchi, K. Tomoda, T. Sawaki, T. Kawanami, M. Tanaka, T. Fukushima, S. Sugai, H. Umehara

Research output: Contribution to journalArticlepeer-review

530 Citations (Scopus)


Background: Mikulicz's disease (MD) has been considered as one manifestation of Sjögren's syndrome (SS). Recently, it has also been considered as an IgG4-related disorder. Objective: To determine the differences between IgG4-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 IgG4-positive multiorgan lymphoproliferative syndrome (IgG4+MOLPS). The preliminary diagnostic criteria include raised serum levels of IgG4 (>135 mg/dl) and infiltration of IgG4+ 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 IgG4+MOLPS than in those with typical SS. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG2, IgG4 and IgE levels were significantly increased in IgG4+MOLPS. Histological specimens from patients with IgG4+MOLPS revealed marked IgG4+ plasma cell infiltration. Many patients with IgG4+MOLPS had lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG4+ 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 IgG4+MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG4+MOLPS.

Original languageEnglish
Pages (from-to)1310-1315
Number of pages6
JournalAnnals of the rheumatic diseases
Issue number8
Publication statusPublished - 2009 Aug
Externally publishedYes

ASJC Scopus subject areas

  • General Biochemistry,Genetics and Molecular Biology
  • Rheumatology
  • Immunology and Allergy
  • Immunology


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