Hypophysitis refers to an
inflammation
Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
of the
pituitary gland
The pituitary gland or hypophysis is an endocrine gland in vertebrates. In humans, the pituitary gland is located at the base of the human brain, brain, protruding off the bottom of the hypothalamus. The pituitary gland and the hypothalamus contr ...
. Hypophysitis is rare and not fully understood.
Signs and symptoms
There are four categories of symptoms and signs. Most commonly, the initial symptoms are headaches and visual disturbances. Some symptoms are derived from the lesser functioning of the adenohypophyseal hormones. Of the adenohypophyseal hormones, the most frequently affected are
corticotropes,
lactotropes and
gonadotrope
1. Introduction
Gonadotropic cells (also known as gonadotropes, gonadotrophs, delta cells, or delta basophils) are endocrine cells in the anterior pituitary that produce gonadotropins. More specifically, gonadotrophs produce and secrete glyco ...
s, all which are found in the anterior pituitary.
Polyuria
Polyuria () is excessive or an abnormally large production or Frequent urination, passage of urine (greater than 2.5 L or 3 L over 24 hours in adults). Increased production and passage of urine may also be termed as diuresis. Polyuria often appe ...
is also a common symptom – which results in very dilute urine, as well as
polydipsia
Polydipsia is excessive thirst or excess drinking.Porth, C. M. (1990). ''Pathophysiology: Concepts of altered health states''. Philadelphia: J.B. Lippincott Company. The word derives , which is derived . Polydipsia is a nonspecific symptom in v ...
which means having extreme thirst. Another symptom is
hyperprolactinemia
Hyperprolactinaemia (also spelled hyperprolactinemia) is a condition characterized by abnormally high levels of prolactin in the blood. In women, normal prolactin levels average to about 13 ng/mL, while in men, they average 5 ng/mL. ...
, which is when there are abnormally high prolactin levels in the blood. Usually, a mass will be found located on the
sella turcica
The sella turcica (Latin for 'Turkish saddle') is a saddle-shaped depression in the body of the sphenoid bone of the human skull and of the skulls of other hominids including chimpanzees, gorillas and orangutans. It serves as a cephalometric la ...
and loss of hormonal function.
Cause
Hypophysitis may have an underlying autoimmune aetiology, as in the case of
autoimmune hypophysitis,
and lymphocytic hypophysitis.
Diagnosis
Mainly, the diagnosis of hypophysitis is through exclusion – patients often undergo surgery because they are suspected of having a pituitary adenoma. But, the most accurate diagnosis is using Magnetic Resonance Imaging (MRI) to find any mass or lesions on the sella turcica.
It is a known side-effect of the new
immune checkpoint
Immune checkpoints are regulators of the immune system. These pathways are crucial for self-tolerance, which prevents the immune system from attacking cells indiscriminately. However, some cancers can protect themselves from attack by stimulat ...
inhibitors of the
CTLA-4
Cytotoxic T-lymphocyte associated protein 4, (CTLA-4) also known as CD152 ( cluster of differentiation 152), is a protein receptor that functions as an immune checkpoint and downregulates immune responses. CTLA-4 is constitutively expressed in ...
inhibitor and
PD-L1 inhibitor classes, used for the treatment of
melanoma
Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye (uveal melanoma). In very rare case ...
, and should be considered in patients on these drugs who present with
endocrine
The endocrine system is a messenger system in an organism comprising feedback loops of hormones that are released by internal glands directly into the circulatory system and that target and regulate distant organs. In vertebrates, the hypotha ...
dysfunction.
Treatment
It was shown through various testing that administration of
bromocriptine
Bromocriptine, originally marketed as Parlodel and subsequently under many brand names, is an ergoline derivative and dopamine agonist that is used in the treatment of pituitary tumors, Parkinson's disease, hyperprolactinaemia, neuroleptic malig ...
can improve field of vision defects and lower
prolactin
Prolactin (PRL), also known as lactotropin and mammotropin, is a protein best known for its role in enabling mammals to produce milk. It is influential in over 300 separate processes in various vertebrates, including humans. Prolactin is secr ...
levels. It was also found that when using
corticosteroids
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...
, there was a decrease in size of the gland, and relieved compression on the dura mater. These corticosteroids were also found to have an immunosuppressive effect which helped with reducing the autoimmune reaction of the gland.
Prognosis
The prognosis for hypophysitis was variable for each individual. The depending factors for hypophysitis included the advancement of the mass on the sella turcica, percentage of fibrosis, and the body's response to corticosteroids. Through the use of corticosteroids, the vision defects tend to recover when the gland size began to decrease. The prognoses of the limited number of reported cases were usually good.
History
The first reported case was in 1962, with a 22-year-old who died of adrenal insufficiency 14 months after giving birth to her second child. Her symptoms began 3 months postpartum, with
lassitude
Fatigue is a state of tiredness (which is not sleepiness), exhaustion or loss of energy. It is a symptom of any of various diseases; it is not a disease in itself.
Fatigue (in the medical sense) is sometimes associated with medical conditions ...
(weakness/lack of energy),
goitre
A goitre (British English), or goiter (American English), is a swelling in the neck resulting from an enlarged thyroid gland. A goitre can be associated with a thyroid that is not functioning properly.
Worldwide, over 90% of goitre cases are ...
(iodine deficiency) and
amenorrhea
Amenorrhea or amenorrhoea is the absence of a menstrual period in a female organism who has reached reproductive age. Physiological states of amenorrhoea are most commonly seen during pregnancy and lactation (breastfeeding). In humans, it is wher ...
(absence of a menstrual period). This was originally reported by Goudie and Pinkerton in Glasgow, UK. There have only been approximately just over 100 cases reported. Majority of these cases were female, and usually began noticing symptoms late into their pregnancies and in early postpartum.
See also
*
Autoimmune hypophysitis
* Lymphocytic hypophysitis
*
Pituitary disease
*
Hypopituitarism
References
{{Pituitary disease
Pituitary disorders
Inflammations