title: A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis.
abstract: BACKGROUND
  Immunoglobulin (Ig) G4-related hypophysitis is an emerging clinical entity, which is characterized by an elevated serum IgG4 concentration and infiltration of IgG4-positive plasma cells in the pituitary. Although some criteria for its diagnosis have been proposed, they have not been fully established. In particular, differential diagnosis from secondary chronic inflammation including granulomatosis with polyangiitis (GPA) is difficult in some cases. We describe central diabetes insipidus with pituitary swelling exhibiting infiltration of IgG4-positive cells.
  
  
  METHODS
  A 43-year-old woman in the remission stage of GPA presented with sudden-onset polyuria and polydipsia. Pituitary magnetic resonance imaging revealed swelling of the anterior and posterior pituitary and stalk, with heterogeneous gadolinium enhancement and disappearance of the high signal intensity of the posterior pituitary. Evaluation of biochemical markers for GPA suggested that the disease activity was well-controlled. Endocrinological examination revealed the presence of central diabetes insipidus and growth hormone deficiency. Pituitary biopsy specimen showed IgG4-positive cells, with a 43% IgG4(+)/IgG(+) ratio, which met the criteria for IgG4-related hypophysitis. However, substantial infiltration of polymorphonuclear neutrophils with giant cells was also noted, resulting in a final diagnosis of pituitary involvement of GPA.
  
  
  CONCLUSIONS
  These results suggest that pituitary involvement of GPA should be taken into account for the differential diagnosis of IgG4-related hypophysitis.
authors.affiliation: Division of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo, 650-0017, Japan.
authors.forename: Hironori
authors.initials: H
authors.lastname: Bando
authors.forename: Genzo
authors.initials: G
authors.lastname: Iguchi
authors.forename: Hidenori
authors.initials: H
authors.lastname: Fukuoka
authors.forename: Masaaki
authors.initials: M
authors.lastname: Taniguchi
authors.forename: Seiji
authors.initials: S
authors.lastname: Kawano
authors.forename: Miki
authors.initials: M
authors.lastname: Saitoh
authors.forename: Kenichi
authors.initials: K
authors.lastname: Yoshida
authors.forename: Ryusaku
authors.initials: R
authors.lastname: Matsumoto
authors.forename: Kentaro
authors.initials: K
authors.lastname: Suda
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nishizawa
authors.forename: Michiko
authors.initials: M
authors.lastname: Takahashi
authors.forename: Akio
authors.initials: A
authors.lastname: Morinobu
authors.forename: Eiji
authors.initials: E
authors.lastname: Kohmura
authors.forename: Wataru
authors.initials: W
authors.lastname: Ogawa
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Takahashi
mesh_terms: D000328:Adult; D000069281:Autoimmune Hypophysitis / Q000175:diagnosis / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D010902:Pituitary Gland / Q000276:immunology*; D011237:Predictive Value of Tests; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G
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