IgG4-related disease

Historical overview and pathology of hematological disorders: Review Article

Yasuharu Sato, Kenji Notohara, Masaru Kojima, Katsuyoshi Takata, Yasufumi Masaki, Tadashi Yoshino

Research output: Contribution to journalArticle

182 Citations (Scopus)

Abstract

IgG4-related diseases comprise a recently recognized systemic syndrome characterized by mass-forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4-positive plasma cells in the affected tissues, and the serum IgG4 level is increased in these patients. The present study describes the history, autoimmune pancreatitis (AIP), IgG4-related lymphadenopathy and lymphomagenesis based upon ocular adnexal IgG4-related disease. Lymphoplasmacytic sclerosing pancreatitis, a prototypal histological type of AIP, is now recognized as a systemic IgG4-related disease. Lymph node lesions can be subdivided into at least five histological subtypes, and systemic IgG4-related lymphadenopathy should be distinguished from multicentric Castleman's disease. Interleukin-6 and CRP levels are abnormally high in multicentric Castleman's disease, but are normal in the majority of systemic IgG4-related lymphadenopathy. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands swelling, and obliterative phlebitis is rare. Moreover, some malignant lymphomas, especially mucosa-associated lymphoid tissue lymphoma, arise from ocular adnexal IgG4-related disease. In addition, IgG4-producing lymphoma also exists.

Original languageEnglish
Pages (from-to)247-258
Number of pages12
JournalPathology International
Volume60
Issue number4
DOIs
Publication statusPublished - Apr 2010

Fingerprint

Immunoglobulin G
Pathology
Pancreatitis
Marginal Zone B-Cell Lymphoma
Lymphoma
Phlebitis
Lacrimal Apparatus
Sclerosis
Plasma Cells
Interleukin-6
Lymph Nodes
History
Serum
Lymphadenopathy

Keywords

  • Autoimmune pancreatitis
  • IgG4
  • Lymph node
  • Mucosa-associated lymphoid tissue lymphoma
  • Ocular adnexa

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

IgG4-related disease : Historical overview and pathology of hematological disorders: Review Article. / Sato, Yasuharu; Notohara, Kenji; Kojima, Masaru; Takata, Katsuyoshi; Masaki, Yasufumi; Yoshino, Tadashi.

In: Pathology International, Vol. 60, No. 4, 04.2010, p. 247-258.

Research output: Contribution to journalArticle

Sato, Yasuharu ; Notohara, Kenji ; Kojima, Masaru ; Takata, Katsuyoshi ; Masaki, Yasufumi ; Yoshino, Tadashi. / IgG4-related disease : Historical overview and pathology of hematological disorders: Review Article. In: Pathology International. 2010 ; Vol. 60, No. 4. pp. 247-258.
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