Mikulicz's disease and systemic IgG4-related plasmacytic syndrome (SIPS)

• YAMAMOTO Motohisa

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• TAKAHASHI Hiroki

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• NAISHIRO Yasuyoshi

  First Department of Internal Medicine, Sapporo Medical University School of Medicine Biomedical Research Center Department of Biomedical Engineering, Sapporo Medical University School of Medicine

• ISSHIKI Hiroyuki

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• OHARA Mikiko

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• SUZUKI Chisako

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• YAMAMOTO Hiroyuki

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

• KOKAI Yasuo

  Biomedical Research Center Department of Biomedical Engineering, Sapporo Medical University School of Medicine

• HIMI Tetsuo

  Department of Otolaryngology, Sapporo Medical University School of Medicine

• IMAI Kohzoh

  Sapporo Medical University

• SHINOMURA Yasuhisa

  First Department of Internal Medicine, Sapporo Medical University School of Medicine

Bibliographic Information

Other Title

• ミクリッツ病と全身性ＩｇＧ４関連疾患　―当科におけるｓｙｓｔｅｍｉｃ　ＩｇＧ４－ｒｅｌａｔｅｄ　ｐｌａｓｍａｃｙｔｉｃ　ｓｙｎｄｒｏｍｅ　（ＳＩＰＳ）　４０例の臨床的検討から―

Abstract

  Mikulicz's disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Both diseases are characterized with elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the glands. Clinical analyses were performed in 40 patients with systemic IgG4-related plasmacytic syndrome (SIPS) who consulted the doctors in Sapporo Medical University Hospital. Our patients were mainly middle-aged or elderly females. The average age was 58.9 years. The diagnosis was following ; 33 cases with Mikulicz's disease, 3 cases with Küttner's tumor, and 4 cases with IgG4-related dacryoadenitis. Slight dysfunction of lacrimal and salivary gland was observed in about 60% of them. Antinuclear antibodies were detected in only 15% of the cases with SIPS. Almost all, except one case, did not have anti-SS-A or anti-SS-B antibodies. Interestingly, hypocomplementemia was revealed in 30% of them. The complications of SIPS include autoimmune pancreatitis, tubulointerstitial nephritis, retroperitoneal fibrosis, prostatitis, and so on. SIPS is mainly treated by the administration of steroids. We started to prescribe much quantity of prednisolone to the patients with organ failure. The recurrence was admitted in the 3 patients for the followed 16 years. We present here the problems and prospects in SIPS.<br>

Journal

• Japanese Journal of Clinical Immunology

  Japanese Journal of Clinical Immunology 31 (1), 1-8, 2008

  The Japan Society for Clinical Immunology

Keywords

• Autoimmune pancreatitis
• Glucocorticoid
• Immune complex
• Mikulicz's disease
• Systemic IgG4-related plasmacytic syndrome (SIPS)

Details

• CRID: 1390001204648534016
• NII Article ID: 10021242142
• NII Book ID: AN00357971
• DOI: 10.2177/jsci.31.1
• COI: 1:CAS:528:DC%2BD1cXotlyjs74%3D
• ISSN: 13497413
• ISSN: 09114300
• ISSN: http://id.crossref.org/issn/09114300
• PubMed: 18311037
• Web Site: http://www.jstage.jst.go.jp/article/jsci/31/1/31_1_1/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2008175456
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • PubMed
  • CiNii Articles
• Abstract License Flag: Disallowed