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.