Lymphocytic autoimmune hypophysitis in a female outside the perinatal period, diagnosed non-invasively and treated successfully

Abstract

Lymphocytic autoimmune hypophysitis (LAH) was first described in 1962, by Goudie and Pinkerton, in Glasgow. It is a neuroendocrine disorder characterised by autoimmune inflammation of the pituitary gland, resulting in various degrees of its dysfunction. In this disorder, the pituitary gland is infiltrated by lymphocytes, which leads to its enlargement and subsequently destruction and impaired function. Typically, the early destruction of ACTH-producing cells is characteristic. LAH is classically observed in young women during pregnancy or postpartum period. We herein report a rare case of lymphocytic autoimmune hypophysitis in a 33 years old non-pregnant female, outside the perinatal period. She presented with an acute left-sided visual loss and a 4-months history of amenorrhea. Her neurological examination revealed the absence of light perception in the left eye. She underwent several investigations including laboratory tests with a hormonal profile, brain MRI, visual evoked potential (VEP), in addition to multiple ophthalmological studies. Her biochemical studies revealed only a low serum cortisol. Her brain MRI findings were suggestive of autoimmune hypophysitis and her VEP revealed a normal P100 latency. She received dexamethasone followed by hydrocortisone, and that consequently lead to a remarkable improvement in both the patient's vision, as well as her follow up MRI brain imaging. We wish to highlight that LAH can present in females outside the perinatal period. Definitive diagnosis of LAH can be achieved only histologically, however, the diagnosis can be made non-invasively if the clinical, radiological, and laboratory investigations are suggestive of it.