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Proposal for a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome: Analysis of 64 cases of IgG4-related disorders.
  1. Yasufumi Masaki (yasum{at}kanazawa-med.ac.jp)
  1. Hematology and Immunology, Kanazawa Medical University
    1. Lingli Dong (pcjjf136{at}yahoo.co.jp)
    1. Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology
      1. Nozomu Kurose (k-nozomu{at}kanazawa-med.ac.jp)
      1. Department of Pathology and Laboratory Medicine, Kanazawa Medical University
        1. Kazuko Kitagawa (kitagawa{at}kanazawa-med.ac.jp)
        1. Department of Ophthalmology, Kanazawa Medical University
          1. Yuko Morikawa (ymjr{at}kanazawa-med.ac.jp)
          1. Department of Epidemiology and Public Health(School of Nursing), Kanazawa Medical University
            1. Motohisa Yamamoto (mocha{at}cocoa.plala.or.jp)
            1. Department of Internal Medicine, Sapporo Medical University School of Medicine
              1. Hiroki Takahashi (htakahas{at}sapmed.ac.jp)
              1. Department of Internal Medicine, Sapporo Medical University School of Medicine
                1. Yasuhisa Shinomura (shinomura{at}sapmed.ac.jp)
                1. Department of Internal Medicine, Sapporo Medical University School of Medicine
                  1. Kohzoh Imai (imai{at}sapmed.ac.jp)
                  1. Sapporo Medical University
                    1. Takako Saeki (saekit{at}nagaoka.jrc.or.jp)
                    1. Department of Internal Medicine, Nagaoka Red-Cross Hospital
                      1. Atsushi Azumi (azumi{at}med.kobe-u.ac.jp)
                      1. Department of Ophthalmology, Department of Surgery, Kobe University Graduate School of Medicine
                        1. Shinji Nakada (insect{at}td5.so-net.ne.jp)
                        1. Department of Japanese Oriental Medicine, University of Toyama
                          1. Eiji Sugiyama (esugiyam{at}nsknet.or.jp)
                          1. First Department of Internal Medicine, University of Toyama
                            1. Shoko Matsui (shoko-tym{at}umin.ac.jp)
                            1. First Department of Internal Medicine, University of Toyama
                              1. Tomoki Origuchi (origuchi{at}net.nagasaki-u.ac.jp)
                              1. Nagasaki Graduate School of Health Sciences
                                1. Susumu Nishiyama (newcity{at}mtd.biglobe.ne.jp)
                                1. Department of Internal Medicine, Kurashiki Medical Center
                                  1. Isao Nishimori (nisao{at}kochi-ms.ac.jp)
                                  1. Department of Gastroenterology and Hepatology, Kochi Medical School
                                    1. Takayuki Nojima (nojima{at}kanazawa-med.ac.jp)
                                    1. Department of Pathology and Laboratory Medicine, Kanazawa Medical University
                                      1. Kazunori Yamada (kyamada50{at}yahoo.co.jp)
                                      1. Department of Internal Medicine, Kanazawa University
                                        1. Mitsuhiro Kawano (kawano{at}lilac.ocn.ne.jp)
                                        1. Department of Internal Medicine, Kanazawa University
                                          1. Yoh Zen (yzen{at}med.m.kanazawa-u.ac.jp)
                                          1. Department of Human Pathology, Kanazawa University
                                            1. Masahiko Kaneko (kaneko{at}uwajima-mh.go.jp)
                                            1. Department of Internal Medicine, Uwajima Hospital
                                              1. Kana Miyazaki (k-myzk{at}clin.medic.mie-u.ac.jp)
                                              1. Department of Hematology and Oncology, Mie University Graduate School of Medicine
                                                1. Kazuo Tsubota (tsubota{at}sc.itc.keio.ac.jp)
                                                1. Department of Ophthalmology, Keio University
                                                  1. Katsumi Eguchi (eguchi{at}net.nagasaki-u.ac.jp)
                                                  1. First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University
                                                    1. Kouichi Tomoda (tomoda{at}kanazawa-med.ac.jp)
                                                    1. Department of Otolaryngology, Kanazawa Medical University
                                                      1. Toshioki Sawaki (toshi-s{at}kanazawa-med.ac.jp)
                                                      1. Hematology and Immunology, Kanazawa Medical University
                                                        1. Takafumi Kawanami (kawahagi{at}kanazawa-med.ac.jp)
                                                        1. Hematology and Immunology, Kanazawa Medical University
                                                          1. Masao Tanaka (masatana{at}kanazawa-med.ac.jp)
                                                          1. Hematology and Immunology, Kanazawa Medical University
                                                            1. Toshihiro Fukushima (tfukus{at}kanazawa-med.ac.jp)
                                                            1. Hematology and Immunology, Kanazawa Medical University
                                                              1. Susumu Sugai (sussugai{at}helen.ocn.ne.jp)
                                                              1. Hematology and Immunology, Kanazawa Medical University
                                                                1. Hisanori Umehara (umehara{at}kanazawa-med.ac.jp)
                                                                1. Hematology and Immunology, Kanazawa Medical University

                                                                  Abstract

                                                                  Background: Mikulicz's disease (MD) has been considered as one manifestation of Sjögren's syndrome (SS). Recently, MD has also been considered as an IgG4-related disorder.

                                                                  Objective: To determine the differences between IgG4-related disorders including MD and SS. Patients and Methods: We investigated patients with MD and IgG4-related disorders registered from all over Japan, and set up provisional criteria for the new clinical entity: IgG4-positive multi-organ lymphoproliferative syndrome (IgG4+MOLPS). The preliminary diagnostic criteria include elevated serum IgG4 (>135 mg/dl) and infiltration of IgG4+ plasma cells in the tissue (IgG4+/IgG+ plasma cells >50%) with fibrosis or sclerosis. We compared clinical features, laboratory data and pathologies of 64 patients with IgG4+MOLPS and 31 patients with typical SS.

                                                                  Results: The incidences of xerostomia, xerophthalmia and arthralgia, rheumatoid factor and anti-nuclear, anti-SS-A/Ro and anti-SS-B/La antibodies were significantly lower in IgG4+MOLPS than typical SS patients. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG2, IgG4 and IgE were significantly elevated in IgG4+MOLPS. Histological specimen from patients with IgG4+MOLPS revealed marked IgG4+ plasma cell infiltration. Many patients with IgG4+MOLPS showed lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG4+ cells were seen in the tissue of typical SS. Thirty-eight patients with IgG4+MOLPS treated with glucocorticoids showed marked clinical improvements.

                                                                  Conclusion: Despite similarities in the involved organs, there are marked clinical and pathological differences between IgG4+MOLPS and SS. Based on the clinical features and good response to glucocorticoid, we propose a new clinical entity: IgG4+MOLPS.

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