A novel concept of Mikulicz's disease as IgG4-related disease

Auris Nasus Larynx. 2012 Feb;39(1):9-17. doi: 10.1016/j.anl.2011.01.023. Epub 2011 May 14.


Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS). However, MD has a unique presentation, including persistent swelling of the lacrimal and salivary glands, and is characterized by good responsiveness to glucocorticoids, leading to recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic "IgG4-related disease." In addition, recent analyses have revealed that Küttner's tumor (KT), a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands, is also associated with prominent infiltration of IgG4-positive plasmacytes. MD and KT differ from SS and are thought to be singular systemic IgG4-related plasmacytic diseases. Here we discuss the results of recent studies and provide an overview of MD as an IgG4-related disease.

Publication types

  • Historical Article
  • Portrait
  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Glucocorticoids / therapeutic use
  • History, 19th Century
  • History, 20th Century
  • Humans
  • Hypergammaglobulinemia*
  • Immunoglobulin G / blood*
  • Lacrimal Apparatus / immunology*
  • Lacrimal Apparatus / pathology
  • Male
  • Middle Aged
  • Mikulicz' Disease / drug therapy
  • Mikulicz' Disease / history
  • Mikulicz' Disease / immunology*
  • Mikulicz' Disease / pathology
  • Parotid Gland / immunology*
  • Parotid Gland / pathology
  • Sialadenitis / immunology
  • Sialadenitis / pathology
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / pathology
  • Submandibular Gland / immunology*
  • Submandibular Gland / pathology


  • Glucocorticoids
  • Immunoglobulin G