Lymphocytic Hypophysitis With Subsequent Development of Graves Disease

Abstract

Lymphocytic hypophysitis (LH), a rare pituitary condition mimicking pituitary macroadenoma, presents most commonly in the peripartum period. This presumably autoimmune disease can occur as an isolated entity or be associated with other autoimmune conditions. The association of LH with Graves disease is extremely rare. We present the clinical course and laboratory evaluation of a woman who developed LH in the third trimester of her fifth pregnancy. The patient then developed overt Graves disease 2 years later. A 21-year-old black woman with an unremarkable medical and obstetric history developed severe headache and partial visual loss during the third trimester of her fifth pregnancy. Evaluation revealed a large partially hemorrhagic pituitary mass with suprasellar extension compressing the optic chiasm. Pathologic evaluation after transsphenoidal surgery confirmed the resected mass to be lymphocytic hypophysitis. Postoperative evaluation documented persistent panhypopituitarism and central diabetes insipidus. She remained clinically stable for 2 years receiving physiological doses of glucocorticoid, levothyroxine, and desmopressin. She then developed symptoms and signs of hyperthyroidism. Levothyroxine was discontinued and additional evaluation was consistent with Graves disease. Patients with severe headache and visual difficulties during pregnancy or the postpartum period should have a detailed neurologic and endocrinologic evaluation. Lymphocytic hypophysitis, a rare pituitary condition, should be considered in the differential diagnosis. It is important to keep close clinical follow up of such patients because they may develop other autoimmune diseases as illustrated by the present case report. (The Endocrinologist 2006;16: 223-226) Learning Objectives• Recall what is known about the demographic and presenting clinical and radiologic features of lymphocytic hypophysitis (LH) as well as its immunologic aspects. • Summarize the hormonal abnormalities found in the present patient and previously reported patients with LH, emphasizing those that make it possible to distinguish between LH and pituitary macroadenoma. • Compare the various treatment options available for patients with LH and the circumstances under which each may be appropriate.L ymphocytic hypophysitis (LH), an uncommon clinical entity, was first described at an autopsy case in 1962. 1 Since then, more than 125 cases of LH have been reported. 2 The majority of patients (approximately 70%) have been young women presenting during pregnancy or in the postpartum period. Approximately 15% of reported patients were men. 2,3 Histologically, LH is characterized by diffuse infiltration of the pituitary by lymphocytes and plasma cells and varying degrees of pituicyte destruction. LH may present as an isolated disorder or be associated with other autoimmune diseases. 2,4,5 LH is thought to have an autoimmune etiology, and the frequent association of LH with pregnancy may be possibly the result of increased exposure to pituitary antigens or changes in maternal ...