| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (NK) Baylor College of Medicine, Houston, Texas |
| Transcript |
"So, today we're going to be talking about LH - Lymphocytic Hypophysitis. It's exactly what it sounds like. It's inflammation (itis) in the hypophysis (in the pituitary gland), and it's infiltrated by lymphocytes. This is an autoimmune process. It's idiopathic. It's associated with postpartum period in young females, late pregnancy, but it can occur in any age, either gender and at any time including the non-pregnant state. So you should be thinking about lymphocytic hypophysitis, however, when we have an infiltrative enlargement of the pituitary gland. ; So as you remember from prior lectures, we have the ventricles here and then the third ventricle, the floor of the third ventricle in coronal section is the chiasm. Then, the infundibulum and in the sella, is the pituitary gland. So the pituitary gland, if it gets bigger, it will rise out of the sella and can compress the chiasm. If it goes laterally, it'll go into the cavernous sinus. And here's the carotid artery and the walls of the cavernous sinus, which means it can cause a third, a fourth, trigeminal or a sixth cranial nerve palsy. So we can either have afferent problems from chiasmal lesion - bitemporal hemianopsia - or efferent problems from diplopia in patients who have sella lesions including lymphocytic hypophysitis. They often present with pan-hypopituitarism, but sometimes they have what looks like a prolactinoma. So they have hyperprolactinemia which means amenorrhea and galactorrhea in young females. However, it might just be the stalk effects. So if you press on the stalk, you are interrupting the dopamine signal from the hypothalamus and that produces an elevation in the prolactin level, leading to the syndrome of amenorrhea and galactorrhea if they have a high enough prolactin level. Often their pan-hypopit, however, they might have infiltration of a stalk which normally would not occur in pituitary adenoma alone. The key differentiating feature is going to be, in the right clinical setting, a pituitary that is enlarged but has an atypical appearance in terms of a lymphocytic infiltrate of appearance, and the treatment is steroids. So as opposed to surgery, transsphenoidal resection for pituitary adenoma, we're going to give this steroids because it's an autoimmune lymphocytic infiltrative process. ; So in the right clinical setting, for example a 24 year old postpartum female who has a new onset bitemporal hemianopsia, the imaging study shows a homogenous infiltrative appearance of lymphocytic hypophysitis. We're going to give that steroids, but the only way to know for sure what the diagnosis is to have a biopsy. And on the biopsy, it's usually a lymphocytic infiltration. You should also know that we have to consider the possibility of IgG4 disease. So, in the past lymphocytic hypophysitis was considered idiopathic, but some of these idiopathic LH cases actually have IgG4 disease. You can look at another video on IgG4 disease to see how that subclass restriction of immunoglobulin class subclass four can infiltrate your pituitary gland. And the reason that's important is this IgG4 infiltration, the ‘itis' can occur in other organ systems - it can cause thyroiditis, it can be retroperitoneal disease, it can be orbital inflammatory pseudotumor. And so the IgG4 just goes to whatever organ system it wants to go and each subspecialty of medicine actually has its own version of orbital inflammatory pseudotumor in their organ. So E&T has it in the thyroid, general surgery has it in the retroperitoneum, every organ has its own version of inflammatory pseudotumor and if it's lymphocytic polyclonal infiltration, you should be thinking about IgG4. ; So in summary, lymphocytic hypophysitis is a disease that usually affects postpartum or pregnant females. It can present with diplopia or vision loss, usually bitemporal hemianopsia. It causes an intrasellar mass with suprasellar extension that can look like pituitary hyperplasia or adenoma. They might have pan-hypopituitarism or hyperprolactinemia, and we should treat that with steroids. If a biopsy is done, make sure it's not IgG4 by testing the IgG4 in the serum but also in the specimen."; ; Summary:; •; Lymphocytic Hypophysitis ; o; An autoimmune, idiopathic , infiltrative enlargement of the pituitary gland ; ; "itis" - inflammation ; ; "hypophysis" - pituitary gland ; ; Infiltrated by the lymphocyte ; o; Etiology ; ; Most commonly associated with postpartum period in young females and late pregnancy but can occur at any age, gender or time (including non-pregnant state); •; Anatomy and Mechanism ; o; The pituitary gland gets bigger, rises out of the sella and compresses in the chiasm in the floor of the third ventricle; ; Pituitary gland can also expand laterally into the cavernous sinus causing a third, fourth, trigeminal or sixth cranial nerve palsy ; •; Presentation and Resulting Problems ; o; Afferent problems like a bitemporal hemianopsia from a chiasmal lesion; o; Efferent problems like diplopia from a sella lesion ; o; Patients may present with pan-hypopituitarism or what looks like a prolactinoma (hyperprolactinemia, amenorrhea and galactorrhea in young females); ; This presentation may be similar to stalk effects. Interrupted dopamine from the hypothalamus elevation in prolactin amenorrhea and galactorrhea ; o; Pituitary will be enlarged with atypical appearance due to homogenous lymphocytic infiltrate ; •; Treatment ; o; Use steroids to treat lymphocytic hypophysitis because it is an autoimmune process; ; Vs. transsphenoidal resection for a pituitary adenoma ; o; Must confirm lymphocytic hypophysitis diagnosis with a biopsy ; ; Consider the possibility of IgG4 disease which can occur in many organ systems ; ; Test for IgG4 in the serum and the specimen |
