IgG4-related disease: historical overview and pathology of hematological disorders

Pathol Int. 2010 Apr;60(4):247-58. doi: 10.1111/j.1440-1827.2010.02524.x.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoantibodies / immunology
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / pathology*
  • Hematologic Diseases / immunology
  • Hematologic Diseases / pathology*
  • Humans
  • Immunoglobulin G / immunology*
  • Lymphatic Diseases / immunology
  • Lymphatic Diseases / pathology
  • Pancreatitis / immunology
  • Pancreatitis / pathology*
  • Plasma Cells / immunology
  • Plasma Cells / pathology*
  • Sclerosis / immunology
  • Sclerosis / pathology
  • Syndrome


  • Autoantibodies
  • Immunoglobulin G