Pituitary Inflammation (Hypophysitis)
Hypophysitis is inflammation of the pituitary gland or pituitary stalk and typically results in varying degrees of pituitary gland failure
There are several types or possible causes of pituitary gland inflammation (also known as hypophysitis). The most common type is lymphocytic hypophysitis followed by granulomatous hypophysitis. Classic lymphocytic hypophysitis occurs predominantly in woman during or after pregnancy, but it can occur in men as well, and across a wide age spectrum. A relatively new form of hypophysitis has recently been described in patients undergoing immunotherapy as a cancer treatment, most commonly in patients with metastatic melanoma being treated with ipilimumab (a human monoclonal antibody against cytotoxic T-lymphocyte antigen 4 ).
Signs and Symptoms of Hypophysitis
The most common symptoms and signs of hypophysitis are anterior and posterior pituitary gland failure (which may include adrenal insufficiency, hypothyroidism, hypogonadism, growth hormone deficiency, as well as diabetes insipidus). Other common symptoms include headache and visual loss.
In some cases, hypophysitis may present with additional symptoms that are specific to certain types or underlying causes. For instance, lymphocytic hypophysitis, which is the most common form, often manifests with symptoms related to autoimmune activity in the body. These symptoms may include fatigue, joint pain, muscle weakness, and general malaise. Other less frequent types of hypophysitis, such as granulomatous hypophysitis or xanthomatous hypophysitis, may have distinct clinical features associated with the nature of the inflammation or the presence of granulomas or lipid-laden cells, respectively. As hypophysitis is a relatively uncommon condition, it is important for individuals experiencing persistent hormonal disturbances, severe headaches, or visual changes to consult a healthcare professional for a comprehensive evaluation. Early diagnosis and proper management can help alleviate symptoms, improve quality of life, and prevent potential complications related to hormonal imbalances or pressure on surrounding structures in the brain.
Diagnosis of Hypophysitis
A presumptive diagnosis of hypophysitis can be made in a patient with otherwise unexplained hypopituitarism with characteristic findings on pituitary MRI of diffuse pituitary gland enlargement and/or a thickened pituitary stalk (infundibulum). In a minority of patients with presumed hypophysitis, a pituitary biopsy may be warranted to confirm the diagnosis if MRI findings are not completely convincing. Pituitary surgery may also be indicated for patients with visual loss from the enlarged pituitary gland impinging upon the optic nerves or optic chiasm. Such a pituitary biopsy can be safely done with an endoscopic endonasal approach.
Treatment of Pituitary Inflammation
Treatment of hypophysitis is typically with hormone replacement therapy and with high dose glucocorticoids (steroids) to reduce inflammation. Despite long-term treatment with steroids, most patients do develop some degree of permanent pituitary gland failure.
At the Pacific Pituitary Disorders Center we have one of the world’s largest and most extensive experiences in diagnosing and treating patients with hypopituitarism of all causes. Our team of pituitary endocrinologists and neurosurgeons work closely together to preserve or restore pituitary gland function and quality of life.
What Causes Hypophysitis
Hypophysitis, an inflammation of the pituitary gland, can result from various causes, affecting hormone production and pituitary function. This rare condition may occur due to autoimmune responses, where the body mistakenly attacks its own pituitary cells, often called lymphocytic hypophysitis. In some patients, medications, especially immune checkpoint inhibitors used in cancer treatments, can trigger hypophysitis as a side effect.
Infections, although less common, can also lead to this inflammation, as can physical trauma or surgery near the pituitary region. Some cases are idiopathic, meaning the cause is unknown.
