Primary Hypophysitis: Idiopathic Inflammatory Disorders of the Pituitary Gland

Summary: Primary hypophysitis is a rare but significant cause of sellar space-occupying lesions. Three forms have been described: granulomatous, lymphocytic, and xanthomatous hypophysitis. Current methods cannot diagnose or distinguish these entities before surgery with sufficient certainty. The disease most commonly presents with sellar space-occupying symptoms as a result of pituitary enlargement and hor-monal abnormalities such as potentially lethal pituitary insufficiency or hyperprolac-tinemia. Pituitary insufficiency almost always ensues, with progressive inflammatory destruction of the pituitary tissue. All three disorders clinically and radiologically mimic pituitary adenomas, and the diagnosis is usually made after surgery. Idiopathic giant cell granulomatous hypophysitis is a cryptogenic inflammatory lesion. The di-agnosis rests on eliminating secondary causes. Lymphocytic hypophysitis is a chronic autoimmune disorder of the anterior and, rarely, posterior pituitary. Although it is most commonly seen in peripartum women, it may be encountered in either sex and may be associated with other autoimmune diseases. Surgical removal, biopsy, and medical therapy are the three options of therapy. Response to medical therapy is unpredictable