Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity

Endocr J. 2009;56(9):1033-41. doi: 10.1507/endocrj.k09e-277. Epub 2009 Nov 19.


Inflammatory lesions of the pituitary gland are rarely encountered. Recently, the concept of immunoglobulin G4 (IgG4)-related systemic disease was proposed in Japan, and more than 20 cases have been reported as possibly associated with infundibulo-hypophysitis since 2000. We herein review such case reports in the published literature and in the abstracts of scientific meetings. Almost all cases involved middle-aged to elderly men presenting with various degrees of hypopituitarism and diabetes insipidus and demonstrating a thickened pituitary stalk and/or pituitary mass. These structures shrank remarkably in response to glucocorticoid therapy, even in a lower dose range similar to that prescribed as a replacement for adrenocortical insufficiency. Some of the anterior pituitary insufficiencies were also resolved by glucocorticoid administration. The presence of IgG4-related systemic disease and an elevated serum IgG4 level before glucocorticoid therapy were the main clues to a correct diagnosis of IgG4-related infundibulo-hypophysitis. Autoimmunity is suggested but not yet established to play a role in the pathogenesis for IgG4-related systemic disease. The fact that hypertrophic pachymeningitis and para-sinusitis accompanied some cases suggested that both sellar and parasellar structures are involved in the chronic inflammation. We therefore classify this disorder not as a variant form of primary autoimmune hypophysitis but as a secondary form of infundibulo-hypophysitis associated with IgG4-related systemic disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Aged
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / diagnosis
  • Diagnosis, Differential
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology*
  • Inflammation / blood
  • Inflammation / complications*
  • Inflammation / diagnosis
  • Male
  • Middle Aged
  • Organ Size
  • Pituitary Diseases / blood
  • Pituitary Diseases / complications*
  • Pituitary Diseases / diagnosis
  • Pituitary Gland* / pathology
  • Pituitary Gland, Posterior* / pathology


  • Immunoglobulin G