Treatment options for primary hypophysitis

What are the natural history and treatment options for primary hypophysitis?

The natural history of hypophysitis typically involves progressive pituitary inflammation, followed by atrophy, fibrosis, and hypopituitarism. Partial spontaneous recovery of both anterior and posterior pituitary function can occur, but persistent hypopituitarism is more common.

In cases of suspected, noninfectious hypophysitis (primary/secondary) and neurosarcoidosis, particularly with symptomatic and progressive disease, an empiric trial of high-dose glucocorticoids (prednisone ≈ 1 mg/kg/day for ∼ 2 to 4 weeks) can be considered, followed by a gradual taper to a physiologic replacement dose. Infectious etiologies should be excluded before committing to a prolonged course of steroids. Glucocorticoids can be effective in reducing mass effects, pituitary/pituitary stalk thickening, and restoring hormone deficiencies, particularly for disease of short duration (< 3–6 months), although randomized, controlled studies that demonstrate clear benefit are lacking. Subsequent steroid dosages and duration are dependent on the disease severity and treatment response. In addition, various other adjuvant immunomodulatory agents, such as rituximab, azathioprine, and methotrexate, have been tried with variable degrees of success.


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