Lymphocytic hypophysitis

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Abstract
The clinical spectrum of lymphocytic hypophysitis is reviewed in 113 cases (96 female and 17 male) of biopsy-proven or suspected disease. Predominantly a disease of young women, the classic acute-phase presentation mimics an enlarging pituitary tumor with partial hypopituitarism or panhypopituitarism in late pregnancy. Distinctive clinical features include isolated corticotropin (ACTH) deficiency and diabetes insipidus, both unusual with pituitary adenoma, and coexistent autoimmune disease. CT and magnetic resonance imaging show a homogeneous, intensely enhancing mass, sometimes involving the pituitary stalk and hypothalamus, termed infundibuloneurohy pophysitis. Subacute disease is increasingly being recognized in the postpartum period, when both spontaneous regression of the mass and hypopituitarism may occur. Neurosurgical intervention often results in permanent hypopituitarism. Steroid therapy is discussed. Chronic disease can mimic Sheehan's syndrome and lead to isolated ACTH deficiency and the empty-sella syndrome. Antipituitary autoantibody testing is reviewed, including an immunoblotting assay that has identified autoantibodies to a 49-kD cytosolic protein in some patients with isolated ACTH deficiency.

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