Infrequent organ involvement of IgG4-related diseases: a literature review

Clin Rheumatol. 2018 May;37(5):1153-1159. doi: 10.1007/s10067-018-4048-8. Epub 2018 Mar 3.


IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD.

Keywords: IgG4-related disease; Infrequent organ involvement; Literature review; Systemic disease.

MeSH terms

  • Autoimmune Diseases / pathology*
  • Humans
  • Immunoglobulin G*
  • Kidney / pathology
  • Lung / pathology
  • Salivary Glands / pathology
  • Skin / pathology
  • Thyroid Gland / pathology


  • Immunoglobulin G