title: An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience.
abstract: Autoimmune hypophysitis is a rare disease with a natural progression that is not well known.
  
  
  
  To collect representative data on clinical features of autoimmune hypophysitis and better characterize the disease.
  
  
  
  A prospective single-center study was designed. Autoimmune hypophysitis-affected patients evaluated from 2011 at our tertiary care Pituitary Unit were enrolled. After ruling out other pituitary masses and secondary causes of hypophysitis, autoimmune hypophysitis was the diagnosis of exclusion. Autoimmune hypophysitis was classified as adenohypophysitis, panhypophysitis, and infundibuloneurohypophysitis according to clinical and neuroradiological findings.
  
  
  
  A total of 21 patients met the inclusion criteria: 9 were diagnosed with adenohypophysitis, 4 with panhypophysitis, and 8 with infundibuloneurohypophysitis. The frequency of secondary hypoadrenalism was similar in adenohypophysitis, panhypophysitis, and infundibuloneurohypophysitis. Growth hormone deficit and secondary hypogonadism occurred more frequently in infundibuloneurohypophysitis than in adenohypophysitis and panhypophysitis (p = 0.009; p = 0.04). All cases of multiple pituitary secretion deficits occurred in cases of infundibuloneurohypophysitis (p = 0.04). No correlations between hypophysitis subtype and anti-pituitary and anti-hypothalamus autoantibodies were found. A higher frequency of extractable nuclear antigens (ENA) and anti-nuclear antibodies (ANA) was found in cases of panhypophysitis (OR 5.0, 95% CI 0.86-28.8, p < 0.001, and OR 1.8, 95% CI 1.1-3.2, p = 0.02, respectively) as compared to adenohypophysitis and infundibuloneurohypophysitis.
  
  
  
  Infundibuloneurohypophysitis should be taken into account in the etiological diagnosis of hypopituitarism, particularly if it is associated with diabetes insipidus and in cases of growth hormone deficit, secondary hypogonadism, or multiple hormone deficits. Contrast-enhanced MRI is crucial for the clinical and noninvasive diagnosis of hypophysitis. Screening for autoantibodies, particularly anti-ENA and anti-ANA, is strongly suggested in the clinical context of hypophysitis.
authors.affiliation: Pituitary Unit, Department of Endocrinology, Catholic University School of Medicine, Rome, Italy.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Chiloiro
authors.forename: Tommaso
authors.initials: T
authors.lastname: Tartaglione
authors.forename: Flavia
authors.initials: F
authors.lastname: Angelini
authors.forename: Antonio
authors.initials: A
authors.lastname: Bianchi
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Arena
authors.forename: Antonella
authors.initials: A
authors.lastname: Giampietro
authors.forename: Marilda
authors.initials: M
authors.lastname: Mormando
authors.forename: Mariacarmela
authors.initials: M
authors.lastname: Sciandra
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Laino
authors.forename: Laura
authors.initials: L
authors.lastname: De Marinis
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000378:metabolism; D034961:Antigens, Nuclear / Q000378:metabolism; D000069281:Autoimmune Hypophysitis / Q000145:classification* / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D010902:Pituitary Gland / Q000000981:diagnostic imaging; D011446:Prospective Studies; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; D034961:Antigens, Nuclear