title: IgG4-related hypophysitis: a new addition to the hypophysitis spectrum.
abstract: BACKGROUND
  Hypophysitis is a chronic inflammation of the pituitary gland that comprises an increasingly complex clinicopathological spectrum. Within this spectrum, lymphocytic and granulomatous hypophysitis are the most common forms, but newer variants have recently been reported.
  
  
  OBJECTIVE
  The aims of the study were to describe a new patient with IgG4-related hypophysitis, review the published literature, and provide diagnostic criteria.
  
  
  METHODS
  A 75-yr-old man presented with a 1-yr history of frontal headache. Initial studies revealed panhypopituitarism and a mass in both the sella turcica and the sphenoidal sinus. The patient underwent transphenoidal surgery, initiated high-dose prednisone followed by hormone replacement therapy, and was closely monitored for 3 yr.
  
  
  RESULTS
  Symptoms improved after prednisone, along with shrinkage of the pituitary and sphenoidal masses, but recurred when prednisone dose was lowered. Histopathology showed a marked mononuclear infiltrate in both the pituitary and sphenoidal specimens, mainly characterized by increased numbers of plasma cells. Many of the infiltrating plasma cells (>10 per high-power field) were IgG4-positive. Review of the literature identified 11 cases of IgG4-related hypophysitis (two diagnosed based on pituitary histopathology).
  
  
  CONCLUSIONS
  We describe the first Caucasian patient with biopsy-proven IgG4-related hypophysitis and provide classification criteria for this disease.
authors: Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
authors: Paola
authors: P
authors: Leporati
authors: Melissa A
authors: MA
authors: Landek-Salgado
authors: Isabella
authors: I
authors: Lupi
authors: Luca
authors: L
authors: Chiovato
authors: Patrizio
authors: P
authors: Caturegli
references: Pituitary. 2012 Jun;15(2):202-8
references: 21479815
references: Endocrinol Jpn. 1986 Aug;33(4):505-10
references: 3792276
references: Endocr Pract. 2007 Mar-Apr;13(2):169-75
references: 17490932
references: Ann Intern Med. 2004 Dec 7;141(11):896-7
references: 15583245
references: Surg Neurol. 2004 Sep;62(3):264-7; discussion 267
references: 15336877
references: Scand J Rheumatol. 2006 Sep-Oct;35(5):410-1
references: 17062446
references: Clin Endocrinol (Oxf). 2010 Jul;73(1):18-21
references: 20039888
references: Neurol Med Chir (Tokyo). 2009 Dec;49(12):622-5
references: 20035143
references: Endocr J. 2008 Aug;55(4):723-8
references: 18493105
references: Endocr J. 2010;57(8):719-25
references: 20467161
references: J Clin Endocrinol Metab. 1993 Jun;76(6):1499-504
references: 8501157
references: Hum Pathol. 2007 Nov;38(11):1720-3
references: 17954209
references: Autoimmun Rev. 2008 Sep;7(8):631-7
references: 18774118
references: J Immunother. 2005 Nov-Dec;28(6):593-8
references: 16224277
references: N Engl J Med. 2007 Apr 12;356(15):1586; author reply 1587
references: 17429095
references: Intern Med. 2011;50(8):931-4
references: 21498945
references: Clin Neuropathol. 2001 Sep-Oct;20(5):229-32
references: 11594509
references: J Invest Dermatol. 2002 Nov;119(5):989-90
references: 12445182
references: Intern Med. 2008;47(12):1139-42
references: 18552474
references: Endocr Rev. 2005 Aug;26(5):599-614
references: 15634713
references: J Zhejiang Univ Sci B. 2009 Jul;10(7):552-8
references: 19585674
references: Pituitary. 2003;6(3):161-8
references: 14971740
references: Adv Anat Pathol. 2010 Sep;17(5):303-32
references: 20733352
references: Endocr J. 2009;56(9):1033-41
references: 19926920
references: Endocr J. 2010;57(6):485-92
references: 20371985
references: Intern Med. 2006;45(21):1243-7
references: 17139126
references: J Gastroenterol. 2003;38(10):982-4
references: 14614606
references: N Engl J Med. 2001 Mar 8;344(10):732-8
references: 11236777
references: J Clin Endocrinol Metab. 2001 Oct;86(10):4603-10
references: 11600510
references: Acta Neuropathol. 2010 Dec;120(6):765-76
references: 20844883
references: J Gastroenterol. 2008;43(6):403-8
references: 18600383
references: Clin Gastroenterol Hepatol. 2006 Aug;4(8):1010-6; quiz 934
references: 16843735