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Hypophysitis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Hypophysitis is a rare condition characterised by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement (1,2,3)

pituitary inflammation can occur as a primary hypophysitis (most commonly lymphocytic, granulomatous or xanthomatous disease) or
secondary hypophysitis (as a result of systemic diseases, immunotherapy or alternative sella-based pathologies)

Caturegli et al. reviewed the disease, showed that lymphocytic hypophysitis is classified into three forms, lymphocytic adenohypophysitis (LAH), lymphocytic infundibulo-neurohypophysitis (LINH), and lymphocytic panhypophysitis (LPH) (2)

adenohypophysitis mainly affects the anterior pituitary
infundibulo-neurohypophysitis involves the posterior pituitary and stalk
panhypophysitis may be heterogeneous, and the pathogenesis includes local inflammation from the anterior pituitary, posterior pituitary, or pathological conditions of hypothalamic-pituitary region such as Rathke's cyst, craniopharyngioma, or germinoma

Traditionally considered a very rare condition, the recent recognition of IgG4-related disease and hypophysitis as a consequence of use of immune modulatory therapy has resulted in increased understanding of the pathophysiology of hypophysitis (1,4)

Hypophysitis usually results in acute and persistent pituitary hormone deficiency requiring long-term replacement (4)

Clinical features:

hypophysitis-related symptoms are:
- headache, disturbances in visual acuity and the visual field, and hypopituitarism due to inflammation and compression of surrounding tissues by the enlarged pituitary
  - adrenocorticotropic hormone (ACTH) secretion is impaired first in most cases of hypophysitis (3)
  - increased prolactin (PRL) levels are observed, resulting in decreased luteinizing hormone/follicle stimulating hormone
  - hypogonadism also occurs by damage of gonadotrophs due to inflammation

Immunological insights:

anti-Rabphilin antibody has been proposed to be a biomarker for lymphocytic infundibulo-neurohypophysitis
immunological disorders such as anti-Pit-1 antibody syndrome are similar to the pathogenesis of lymphocytic hypophysitis - congenital combined TSH, GH, and PRL deficiency due to a Pit-1 gene mutation is a well-known cause of short stature
immune checkpoint blockade such ipilimumab has been shown to induce anti-CTLA-4-related hypophysitis

Management of hypophysitis includes control of the inflammatory pituitary mass using a variety of treatment strategies including surgery and medical therapy

glucocorticoids remain the mainstay of medical treatment but other immunosuppressive agents (e.g. azathioprine, rituximab) show benefit in some cases, but there is a need for controlled studies to inform practice (4)

Reference:

Lin HH et al. In situ activation of pituitary-infiltrating T lymphocytes in autoimmune hypophysitis. Sci Rep 2017; 7: 43492.
Caturegli P et al. Autoimmune hypophysitis. Endocr Rev 2005; 26: 599-614.
Hashimoto K, Takao T, Makino S: Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J 1997; 44: 1-10.
Joshi MN et al.MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment. Eur J Endocrinol. 2018 Sep;179(3):R151-R163

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