Putative IgG4-related pituitary disease with hypopituitarism and/or diabetes insipidus accompanied with elevated serum levels of IgG4

Endocr J. 2010;57(8):719-25. doi: 10.1507/endocrj.k10e-030. Epub 2010 May 13.

Abstract

IgG4-positive plasma cell infiltration into multiple organs or tissues, such as the pancreas and salivary glands, associated with increased serum levels of IgG4 is a characteristic finding seen in IgG4-related disease. Affected organs may appear tumorous as a result of chronic inflammatory processes accompanied with progressive fibrosis. Recent cases of this disorder in which the pituitary gland was affected include cases of diffuse enlargement of the pituitary and/or its stalk associated with central diabetes insipidus and/or impaired anterior hormone production. Here we report two such cases, as well as two additional previously undiagnosed cases found in our database. In order to make a correct diagnosis of pituitary lesion involvement with IgG4-related disease, the clinical background and concomitant disorders should be carefully taken into consideration and the measurement of serum levels of IgG4 seems to be useful.

Publication types

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

MeSH terms

  • Aged
  • Diabetes Insipidus / complications
  • Diabetes Insipidus / diagnosis
  • Diabetes Insipidus / immunology*
  • Diagnosis, Differential
  • Female
  • Humans
  • Hypopituitarism / complications
  • Hypopituitarism / diagnosis
  • Hypopituitarism / immunology*
  • Immunoglobulin G / blood*
  • Magnetic Resonance Imaging
  • Male
  • Pituitary Hormones / blood
  • Prednisolone / therapeutic use

Substances

  • Immunoglobulin G
  • Pituitary Hormones
  • Prednisolone