title: Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women.
abstract: IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients with no evidence of involvement of other organs at the time of diagnosis.
  
  
  CONCLUSIONS
  IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis.It is more common in older men, but young women may also present this type of hypophysitis.Although involvement of other organs is frequent, isolated pituitary disease is possible.Frequent clinical manifestations include anterior hypopituitarism and/or diabetes insipidus.THE DIAGNOSIS MAY BE CONFIRMED WITH ANY OF THE FOLLOWING CRITERIA: a pituitary biopsy with lymphoplasmacytic infiltrates, with more than ten IgG4-positive cells; a sellar mass and/or thickened pituitary stalk and a biopsy-proven involvement of another organ; a sellar mass and/or thickened pituitary stalk and IgG4 serum levels >140 mg/dl and sellar mass reduction and symptom improvement after corticosteroid treatment.Glucocorticoids are recommended as first-line therapy.
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Gabriela Alejandra
authors.initials: GA
authors.lastname: Sosa
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Soledad
authors.initials: S
authors.lastname: Bell
authors.affiliation: Department of Pathology, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Silvia Beatriz
authors.initials: SB
authors.lastname: Christiansen
authors.affiliation: Department of Radiology, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Marcelo
authors.initials: M
authors.lastname: Pietrani
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Mariela
authors.initials: M
authors.lastname: Glerean
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Monica
authors.initials: M
authors.lastname: Loto
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Soledad
authors.initials: S
authors.lastname: Lovazzano
authors.affiliation: Department of Neurosurgery, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Antonio
authors.initials: A
authors.lastname: Carrizo
authors.affiliation: Department of Neurosurgery, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Pablo
authors.initials: P
authors.lastname: Ajler
authors.affiliation: Department of Endocrinology, Metabolism and Nuclear Medicine, Hospital Italiano , Perón 41901202, Buenos Aires , Argentina.
authors.forename: Patricia
authors.initials: P
authors.lastname: Fainstein Day