From the Guidelines
Hypophysitis is inflammation of the pituitary gland, which can occur in several forms, including lymphocytic, granulomatous, xanthomatous, and IgG4-related hypophysitis, and can also develop as a side effect of immune checkpoint inhibitors used in cancer treatment. According to the most recent and highest quality study 1, hypophysitis is characterized by inflammation of the pituitary gland, leading to hormonal deficiencies, and can present with symptoms such as fatigue, low blood pressure, nausea, headaches, and vision changes.
Causes and Risk Factors
The condition can occur in several forms, including lymphocytic hypophysitis, granulomatous hypophysitis, xanthomatous hypophysitis, and IgG4-related hypophysitis, and can also develop as a side effect of certain medications, particularly immune checkpoint inhibitors used in cancer treatment, as noted in 1.
Diagnosis and Treatment
Diagnosis usually involves blood tests to assess hormone levels, MRI imaging of the brain, and sometimes a biopsy, as recommended in 1. Treatment depends on the cause and severity but often includes hormone replacement therapy to address deficiencies, such as hydrocortisone for adrenal insufficiency, levothyroxine for hypothyroidism, or sex hormones. In cases related to autoimmune processes or medication side effects, corticosteroids like prednisone may be prescribed to reduce inflammation, as suggested in 1.
Management and Education
All patients with hypophysitis should receive education on stress dosing for sick days, use of emergency injectables, and a medical alert bracelet for adrenal insufficiency to trigger stress-dose corticosteroids by emergency medical personnel, as emphasized in 1 and 1. Endocrine consultation should be part of planning before surgery or high-stress treatments, and patients should be instructed to obtain and carry a medical alert bracelet, as noted in 1.
Key Considerations
It is essential to start corticosteroids first when planning hormone replacement therapy for multiple deficiencies, as other hormones can accelerate the clearance of cortisol and precipitate adrenal crisis, as warned in 1. Additionally, patients with adrenal insufficiency should be instructed to obtain and carry a medical alert bracelet to trigger stress dose corticosteroids by EMS, as recommended in 1 and 1.
From the FDA Drug Label
YERVOY can cause immune-mediated hypophysitis Hypophysitis can present with acute symptoms associated with mass effect such as headache, photophobia, or visual field cuts. Hypophysitis can cause hypopituitarism.
Hypophysitis is an immune-mediated condition that can cause hypopituitarism and present with symptoms such as headache, photophobia, or visual field cuts due to mass effect 2.
From the Research
Definition of Hypophysitis
- Hypophysitis is a rare condition characterized by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement 3.
- It can be classified as primary hypophysitis (most commonly lymphocytic, granulomatous or xanthomatous disease) or secondary hypophysitis (as a result of systemic diseases, immunotherapy or alternative sella-based pathologies) 3.
Causes and Forms of Hypophysitis
- Hypophysitis can occur as a result of various factors, including autoimmune disorders, infections, and the use of immune checkpoint inhibitors 4, 5.
- IgG4-related hypophysitis and immune checkpoint inhibitor-induced hypophysitis are two novel forms of hypophysitis that have been increasingly recognized 4, 5.
- Lymphocytic hypophysitis is the most common cause of primary hypophysitis, while histiocytosis and granulomatous diseases are the most frequent causes of secondary hypophysitis 5.
Clinical Presentation and Diagnosis
- The clinical presentation of hypophysitis varies from asymptomatic to fatal disease, often as a result of electrolyte abnormalities due to glucocorticoid deficiency in the context of adrenal crisis from central adrenal insufficiency 4.
- Non-invasive diagnosis of hypophysitis remains elusive, and the use of currently available serum anti-pituitary antibodies is limited by low sensitivity and specificity 3.
- Modern imaging techniques, histological classification, and immune profiling are improving the accuracy of the diagnosis of hypophysitis 3.
Management and Treatment
- Management of hypophysitis includes control of the inflammatory pituitary mass using a variety of treatment strategies, including surgery and medical therapy 3.
- Glucocorticoids remain the mainstay of medical treatment, but other immunosuppressive agents (e.g. azathioprine, rituximab) show benefit in some cases 3.
- Timely diagnosis and intervention are important to minimize disease-related morbidity and mortality 4, 5.