Lymphocytic hypophysitis. A review of 145 cases.

Lymphocytic hypophysitis (LH) is a new clinico pathological entity, first described in 1962 by Goudie [46]. With recent advances in radiological investigation and technical refinements of trans-sphenoidal surgery, LH cases are being reported with increasing frequency. A total of 145 cases have been reported to date, 102 of them since 1990, with 121 histologically proven. LH is an autoimmune endocrinopathy characterized by an extensive infiltration of the anterior pituitary gland by chronic inflammatory cells, which can cause pituitary expansion and a variable degree of hypopituitarism closely mimicking the features observed in pituitary adenoma. Involvement of the neurohypophysis rarely occurs in LH. The disease mostly occurs at the end of gestation or during the early post partum, but not exclusively: 23 cases of men and 13 cases of menopausal women have been reported in the literature. When no diagnosis is made, the sometimes insidious clinical course can progress towards hypopituitarism and can result in a lethal outcome. Better known since the last five years, clinical management remains a question of debate. Up to now, the majority of cases of adenohypophysitis were surgically treated leading to severe secondary endocrine deficiencies. Recently some authors (including in one personal case report) reported a new approach with corticosteroid therapy, which was successful in reversing the hypophy-seal mass and the pituitary endocrine insufficiency. Lymphocytic hypophysitis is a recently individualized autoimmune endocrinopathy, similar to Hashimoto's thyroi-ditis. The frequency of the disease is probably underestimated and some cases of idiopathic pituitary insufficiency of adults or primary idiopathic empty sella syndrome might be related to previously ignored LH. Pathological definition