IgG4–related systemic disease

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IgG4–related systemic disease (IgG4-RSD), also known as hyper-IgG4 disease and IgG4-related disease is a disease in which inflammatory cells cause fibrosis, the deposition of connective tissue, in one or more organs. The disease is so named because the antibody subtype IgG4 can be detected on tissue samples and often at elevated levels in the bloodstream. The association with IgG4 is a relatively recent finding, and the condition has been described under numerous other names in the past. Diseases such as autoimmune pancreatitis, retroperitoneal fibrosis, mediastinal fibrosis, Riedel's thyroiditis, Mikulicz's syndrome, Kuttner's tumor and inflammatory pseudotumor are now regarded as forms of IgG4-RSD.[1][2]

IgG4-RSD is characterised pathologically by three histopathological features, which are: storiform pattern of fibrosis, lymphoplasmocytic infiltrate, and obliterative phlebitis.[3]

IgG4-RSD is mentioned in the medical literature under different names:[4]

  • IgG4-related autoimmune disease
  • IgG4-associated multifocal systemic fibrosis
  • IgG4-related systemic disease (IgG4-RSD)
  • IgG4-related sclerosing disease
  • Hyper-IgG4 disease
  • IgG4-related disease (IgG4-RD)
  • Systemic IgG4 plasmacytic syndrome (SIPS)
  • IgG4-related multi-organ lymphoproliferative syndrome (IgG4-MOLPS)
  • IgG4-associated disease


  1. ^ Khosroshahi A, Stone JH (Jan 2011). "A clinical overview of IgG4-related systemic disease". Curr Opin Rheumatol 23 (1): 57–66. doi:10.1097/BOR.0b013e3283418057. PMID 21124086. 
  2. ^ Stone JH (February 2012). "IgG4-Related Disease". New England Journal of Medicine 336 (6): 539–51. doi:10.1056/NEJMra1104650. PMID 22316447. 
  3. ^ Deshpande, Vikram (18 May 2012). "Consensus statement on the pathology of IgG4-related disease". Modern Pathology 25: 1181–1192. doi:10.1038/modpathol.2012.72. PMID 22596100. 
  4. ^ Umehara H, Okazaki K, Masaki Y, et al. (2012). "A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details". Mod Rheumatol 22 (1): 1–14. PMID 21881964.