The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity

Autoimmun Rev. 2010 Jul;9(9):591-4. doi: 10.1016/j.autrev.2010.05.003. Epub 2010 May 10.


IgG4-related disease is a distinct clinical entity, whose characteristic features are the following; Serum IgG4 is prominently elevated, IgG4-positive plasma cells infiltrate in involved tissues, various mass-forming lesions with fibrosis develop in a timely and spatial manner and the response to corticosteroids is prompt and good. IgG4-related diseases mainly target two organs. One is the pancreas (autoimmune pancreatitis; AIP), and the other comprises the lacrimal and salivary glands, the clinical phenotype is Mikulicz's disease (MD). MD has long been considered a manifestation of Sjögren's syndrome (SS). However, we noticed several clinical differences in case of MD from SS; no deflection of female sex differences, mild sicca syndrome, good response to corticosteroids, no positivity of anti-SS-A/SS-B antibodies. In addition, elevated level of serum IgG4 and abundant infiltration of plasma cells expressing IgG4 were reported in MD patients. Those are common features of IgG4-related diseases. MD often coexisted with IgG4-related diseases such as AIP, retroperitoneal fibrosis, and IgG4-associated nephropathy. Based on those findings, it has been considered to recognize IgG4-related diseases including MD as a new clinical entity. The etiology of IgG4-related systemic diseases remains to be elucidated. It is necessary to accumulate and analyze larger data from patients worldwide.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / pathology
  • Humans
  • Immune System Diseases / diagnosis*
  • Immunoglobulin G / blood*
  • Lacrimal Apparatus / pathology
  • Mikulicz' Disease / diagnosis
  • Mikulicz' Disease / immunology
  • Mikulicz' Disease / pathology
  • Pancreatitis / diagnosis
  • Pancreatitis / immunology
  • Pancreatitis / pathology
  • Salivary Glands / immunology
  • Salivary Glands / pathology
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / pathology
  • Syndrome


  • Immunoglobulin G