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 journalReview articlepeer-review

220 Citations (Scopus)


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
Issue number4
Publication statusPublished - Apr 2010


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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


Dive into the research topics of 'IgG4-related disease: Historical overview and pathology of hematological disorders: Review Article'. Together they form a unique fingerprint.

Cite this