IgG4-related diseases

Best Pract Res Clin Rheumatol. 2012 Aug;26(4):425-38. doi: 10.1016/j.berh.2012.07.001.


Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fascinating condition recognised as a systemic disease in 2003 [1,2]. The first link between autoimmunity affecting the pancreas, elevated serum IgG4 concentrations and large numbers of IgG4-positive plasma cells in pancreatic tissue was described only 2 years earlier [3]. Since then, many diseases that have long been viewed organ-specific are now considered within the spectrum of IgG4-RD. Practically any organ can be affected, having in common a key pathological feature consisting in dense lymphocyte and plasma cell infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and often an elevated serum IgG4 concentration. While good clinical response to steroid therapy is observed, immunosuppressive or B-cell depleting therapy can be required. It is important to distinguish the IgG4-RD from traditional organ-specific autoimmune disease to guide therapy.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Female
  • Humans
  • Immunoglobulin G / immunology*
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Organ Specificity / immunology
  • Plasma Cells / immunology
  • Plasma Cells / pathology
  • Rare Diseases / diagnosis
  • Rare Diseases / drug therapy
  • Rare Diseases / immunology*
  • Rare Diseases / pathology
  • Sex Factors


  • Immunoglobulin G
  • Immunosuppressive Agents