Lymphocytic Hypophysitis

Abstract

Lymphocytic hypophysitis (LH) is an autoimmune endocrine disorder primarily affecting peripartum young women. Lymphocytic infiltration of the pituitary initially results in pituitary enlargement, which can cause symptoms and radiographic evidence of a mass lesion. Partial or panhypopituitarism may be present. The anterior pituitary is usually affected, although diabetes insipidus can occur. Over time, the pituitary undergoes fibrosis and atrophy; radiography can demonstrate a partial to empty sella. Most patients will have permanent hypopituitarism, but recovery of function has been documented. In the absence of compressive symptoms such as headache or visual impairment, conservative management is recommended because the initial mass lesion seen with LH could spontaneously regress. Hormone replacement therapy should be given as needed. If there are significant neurologic symptoms secondary to a mass effect, a short course of glucocorticoids can be initiated. Further progression of neurologic symptoms warrants neurosurgical intervention. Partial decompression can relieve symptoms while avoiding removal of significant amounts of pituitary tissue. Patients should be monitored for recovery of pituitary function. The incidence of recurrence of LH with future pregnancies is unknown. We report a case of a woman with LH documented by histology who became spontaneously pregnant several years later without recurrence of lymphocytic hypophysitis.