Lymphocytic hypophysitis
Lymphocytic hypophysitis is a presumed autoimmune inflammation of the pituitary gland that typically presents in women during pregnancy or the postpartum period, with a sellar mass, hyperprolactinemia, or hypopituitarism. There is no specific therapy; management is hormonal replacement of any deficient axis as needed, with surgery reserved for mass effect that does not respond to observation.
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What this article covers
- Overview
Definition: lymphocytic hypophysitis is a presumed autoimmune inflammatory infiltration of the pituitary gland by lymphocytes, producing a sellar mass, hyperprolactinemia, or hypopituitarism.
- Epidemiology
Occurs in adults, predominantly women: the female predominance is pronounced, consistent with its presumed autoimmune basis.
- Risk factors
Female sex. Pregnancy, particularly the third trimester, and the early postpartum period: this is the dominant clinical setting in which the disease presents.
- Pathophysiology
Lymphocytic infiltration of the pituitary gland is presumed to be autoimmune, analogous mechanistically to other lymphocyte-mediated autoimmune endocrinopathies with which it clusters.
- Clinical features
Presentation may be a sellar mass with associated hyperprolactinemia, clinically resembling a prolactinoma.
- Differential diagnosis
Prolactinoma: also presents with a sellar mass and hyperprolactinemia in a woman of reproductive age, but prolactinoma is a discrete neoplasm rather than diffuse lymphocytic infiltration, and this is…
- Diagnosis
The diagnosis depends on the clinical associations (pregnancy or postpartum timing, coexisting autoimmune endocrinopathy) together with the results of surgical exploration, since the sellar…
- Management
First, assess and treat any hormone deficiency, since no specific therapy targets the underlying inflammation itself: hormonal replacement is given as needed for whichever axis is deficient.
- Complications
Permanent hypopituitarism, particularly persistent ACTH deficiency requiring lifelong glucocorticoid replacement.
- Prognosis
Course is variable: some patients have a self-limited process with partial or full recovery of pituitary function, while others develop permanent hormone deficiencies.
- Prevention
No primary prevention exists because the triggering autoimmune process is not modifiable.
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