title: Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders.
abstract: BACKGROUND
  Mikulicz's disease (MD) has been considered as one manifestation of Sjögren'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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa, 920-0293, Japan. yasum@kanazawa-med.ac.jp
authors.forename: Y
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authors.lastname: Masaki
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authors.lastname: Saeki
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authors.lastname: Sugiyama
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authors.lastname: Origuchi
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authors.lastname: Miyazaki
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authors.lastname: Eguchi
authors.forename: K
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authors.lastname: Tomoda
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authors.initials: T
authors.lastname: Sawaki
authors.forename: T
authors.initials: T
authors.lastname: Kawanami
authors.forename: M
authors.initials: M
authors.lastname: Tanaka
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authors.lastname: Fukushima
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authors.lastname: Sugai
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authors.lastname: Umehara
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007765:Lacrimal Apparatus / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D012470:Salivary Glands, Minor / Q000473:pathology; D012859:Sjogren's Syndrome / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D013577:Syndrome; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone