Lymphocytic hypophysitis

Abstract: Lymphocytic hypophysitis (LH) is an inflammatory disease of the anterior pituitary. The varying clinical presentation and the short-term outcome of LH have been extensively described in several case reports or small cohort studies. However, little is known about the long-term outcome of this disease. It is currently believed that if left untreated it may run a self-limited course followed by full resolution of the mass with or without persisting pituitary failure. We describe a 29-yr-old female who presented with secondary amenorrhea, headaches, visual defects, and a pituitary mass, which was removed by transsphenoidal surgery. Histology was consistent with the diagnosis of LH. Following surgery the patient demonstrated a gradual recovery of gonadotroph function with restoration of menses and a successful pregnancy. However, 3 yr after delivery and 6 yr following her initial presentation she developed amenorrhea, headaches, and a pituitary mass. Institution of steroid therapy resulted in resolution of the pituitary mass. In summary, this case illustrates that similarly to many other disorders of autoimmune origin LH may run a fluctuating course and late recurrence is possible even after the successful removal of the inflammatory mass, thus necessitating long-term follow-up of these patients.