An Overview of Hypophysitis

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Hypophysitis is a rare condition which causes inflammation of the pituitary gland, an important hormone-producing gland in the brain. There are two main types of hypophysitis: classified according to its etiology. primary (isolated inflammation of the gland not associated with any other disorder), or secondary, as a result of systemic diseases, infections, or drug-induced.

Hypophysitis can also be classified according to the cells that cause the inflammation, including: lymphocytic, granulomatous, xanthomatous, and plasmacytic. Even though these conditions look different on a cellular level, they often share similar symptoms.

Lymphocytic hypophysitis is the most frequent type, affecting women more than men, and frequently occurs at the end of pregnancy or during the postpartum period. Granulomatous and xanthomatous hypophysitis are also more common in women, but not linked to pregnancy, while the plamacytic type is more common in older men.

If hypophysitis is not managed or controlled, this condition may contribute to the development of hypopituitarism, which is decreased pituitary gland function.

Symptoms

Symptoms related to all types of hypophysitis include frequent headaches, vision changes (either low vision or double vision), and impaired pituitary function.

Most of the hormones secreted by the anterior portion of the pituitary gland including ACTH, TSH, growth hormone, and sexual hormones are usually decreased in hypophysitis, while prolactin levels can be low or high. If the posterior portion of the gland or/and the pituitary stem are involved, diabetes insipidus can occur.

The deficiency of the above hormones can cause a variety of symptoms, including a decrease in the body's reaction to stress (ACTH), fatigue and cold intolerance (TSH), sexual dysfunction, and infertility (sexual hormones). If the decrease in ACTH is severe enough, it can be fatal. Low prolactin is associated with decreased milk production, while high levels can cause infertility, lack of or abnormal menstrual periods, and galactorrhea (an increase in milk production). Diabetes insipidus is associated with excessive thirst and increased urination due to a deficiency of antidiuretic hormone.

Additionally, hypophysitis stops production of adrenocorticotropic hormone (ACTH), which plays a role in essential bodily functions such as the release of another hormone called cortisol. Both cortisol and ACTH influence the body’s stress response, appetite, sleep, and the functions of each organ. If this lack of ACTH and cortisol is severe enough, this may be fatal.

Secondary symptoms which result from the changes in hormone levels include increased urination and excessive thirst as diabetes insipidus develops. Sexual dysfunction (including low drive in both sexes along with erectile dysfunction in men), weight loss, fatigue, and low levels of sodium in the blood (called hyponatremia) may be seen. Additional symptoms which are specific to granulomatous inflammation include nausea, vomiting, and an excessive production of the hormone prolactin.

Causes

Hypophysitis occurs when the pituitary gland is attacked by different types of cells including lymphocytes, plasma cells, giant cells, and histiocytes. While scientists have not determined why the body assumes this response toward the pituitary gland, many consider this condition to be an autoimmune disease. Autoimmune dysfunction is the result of the body’s immune system attacking its own cells. This is dangerous and can cause a variety of symptoms, depending on the organ affected.

Hypophysitis can also occur in infections such as tuberculosis, syphilis, and fungal infection, and more recently has been detected in patients undergoing immunotherapy for cancer, especially with ipilimumab monoclonal antibody used to treat metastatic melanoma.

There is some research indicating an autoimmune cause is correct, since this condition commonly occurs in women who have recently given birth and often have unbalanced hormone levels. Specifically, lymphocytic hypophysitis has been noted to occur more frequently in women than men, due to its connection with a history of women who are in later pregnancy or who have recently given birth. Despite the increased occurrence in this population, hypophysitis may also occur in women with no history of pregnancy and men.

Diagnosis

A diagnosis of hypophysitis is made by completing laboratory tests to confirm the abnormal hormone levels in the blood and by imaging studies, usually magnetic resonance imagining (MRI) with contrast. MRI abnormalities may include diffuse enlargement and/or homogeneous contrast enhancement of the pituitary gland, as well as thickening of the pituitary stem.

In cases of secondary hypophysitis, additional tests will be needed depending on the suspected etiology.

It is important that your physician first rules out the presence of pituitary masses, infectious diseases, and other inflammatory diseases affecting larger areas or body systems. Not only will this ensure an accurate diagnosis, but confirmation will assist in providing the appropriate treatment methods.

Radiographic imaging (often done by magnetic resonance imaging, or MRI) is also often completed to verify the tissue changes affecting the pituitary gland, which often appears as sparse tissue with areas of clear tissue break down.

Surgery may be required to obtain tissue for a biopsy. This will confirm a diagnosis of hypophysitis and rule out any cancerous activity present in the pituitary gland. If the gland is enlarged and the pituitary stalk or mucosa is thick, this could point toward a diagnosis of hypophysitis.

It is often easier to make a diagnosis of hypophysitis in pregnant women due to the regular and extensive hormone checks which these women undergo. It is often unnecessary to confirm hypophysitis through radiographic imaging for these women, though it is able to be done if needed. For those instances where radiographic imaging is not completed to confirm a diagnosis of hypophysitis, it is strongly advised that individuals receive closer monitoring and follow-up after treatment.

Treatment

Hypophysitis treatment is typically recommended if symptoms are largely impacting function or are painful. This includes severe headaches and vision changes which are due to compression of the optic nerve (which will cause blindness if left unmanaged).

Treatment of hypophysitis using immunosuppressive medications is indicated in some cases, though this is at the discretion of your physician and according to your circumstances. It remains unclear whether the use of immunosuppressive medications is more effective at treating hypophysitis than other treatments. However, it is typically not recommended that individuals remain on immunosuppressive medication regimens for long periods of time. Hormone replacement therapy is also needed.

Surgery is reserved for severe cases in which there is significant enlargement of the pituitary gland with compression of nearby structures.

Some cases of hypophysitis spontaneously resolve, which eliminates the need for any treatment outside of intermittent monitoring to decrease the risk of recurrence. A German research study examined the progress of 22 cases of hypophysitis. Most patients received a temporary course of glucocorticoid steroids and received either partial or complete surgical removal of the pituitary gland. Radiographic results showed 16 of the 22 cases resolved or entered a stable state after completing these short term treatments followed by a period of monitoring. Many individuals have success with treating headaches related to hypophysitis. However, such steroid medications often cause side effects and an increased risk of symptoms returning.

A German retrospective research study involving 76 patients with hypophysitis showed that many patients with milder forms of the disease responded well to treatment. However, patients with more severe or progressive forms of disease required steroids and/or surgery. Steroids were initially quite effective, but symptoms tended to recur after treatment was tapered or discontinued. Side effects from steroids were also common. Surgery was beneficial in clarifying the diagnosis and relieving symptoms, but its impact on the long-term course of hypophysitis is still unclear.

A Word From Verywell

Hypophysitis is a rare, yet manageable condition involving inflammation of the pituitary gland. While it may cause symptoms which impact quality-of-life and function, many individuals do not experience largely impactful symptoms. Such individuals are able to cope with this condition by receiving monitoring and an official diagnosis to ensure the condition is managed properly.

If you are experiencing symptoms which have a negative impact on your life, you should consult your doctor regarding treatment options. Your doctor will be able to complete a physical exam, assess your medications, medical history, family history, general health, and complete tests such as blood draws and radiographic exams to determine what option is best for you. Despite receiving treatment, some symptoms many not entirely resolve. As always, maintaining a positive outlook and seeking support with assistance for your mental health and emotional response to this condition is of vast importance.

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