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lymphocytic (autoimmune) hypophysitis
Etiology:
1) autoimmune in association with other autoimmune endocrinopathies
2) generally occurs during or after pregnancy [2]
3) immune checkpoint inhibitors
- nivolumab, pembrolizumab, ipilimumab, tremelimumab [2]
Epidemiology:
1) rare
2) affects adults
- cancer patients treated with immune checkpoint inhibitor [2]
3) predominantly affects women, especially during pregnancy & post-partum
Pathology:
1) pituitary infiltration with lymphocytes & plasma cells
2) fibrosis & hypopituitarism
3) symmetrical enlargement of the sellar contents
4) anti-pituitary antibodies (not routinely measured)
Clinical manifestations:
1) hypopituitarism
- ACTH deficiency resulting in adrenal insufficiency
- hypotension
2) hyperprolactinemia may occur
3) diabetes insipidus may occur
4) symptoms of a mass lesion including visual defects may occur
Laboratory:
- serum ACTH, serum cortisol (AM cortisol)
- serum TSH, serum T4, free T4
- serum IGF1
Radiology:
- MRI may show homogeneous & symmetric mass of the sella turcica [2]
Complications:
- adrenal insufficiency is a major cause of morbidity & mortality [2,3]
- visual field defects
Differential diagnosis:
1) prolactinoma
2) Sheehan's syndrome
Management:
1) neurosurgery
a) exploration may be needed for diagnosis
b) visual field defects
2) hormone replacement as needed
a) hydrocortisone
b) thyroxine
c) estrogen
d) growth hormone
3) prognosis:
- size of pituitary mass often decreases over time [2]
Related
diabetes insipidus (DI)
hyperprolactinemia
hypopituitarism
pituitary adenoma/prolactin secreting (prolactinoma)
sella turcica (hypophyseal fossa)
Sheehan's syndrome
General
hypophysitis
autoimmune disease
pregnancy disorder; obstetric disorder; pregnancy complication
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 197
- Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 19.
American College of Physicians, Philadelphia 1998, 2009, 2012, 2022
- Medical Knowledge Self Assessment Program (MKSAP) 19
Board Basics. An Enhancement to MKSAP19.
American College of Physicians, Philadelphia 2022
- Molitch ME, Gillam MP.
Lymphocytic hypophysitis.
Horm Res. 2007;68 Suppl 5:145-50
PMID: 18174733