Abstract
BackgroundAutoimmune hypophysitis is a rare condition that often results in enlargement of the pituitary gland and hypopituitarism due to inflammatory infiltration. Management of autoimmune hypophysitis can include long-term hormonal replacement and close control of the inflammatory pituitary mass. Mass-related symptoms in patients with autoimmune hypophysitis are treated with anti-inflammatory therapy, surgery, and/or radiotherapy.Case presentationWe present a 25-year-old White man with visual field defects of the right eye, headache, and weight loss. Magnetic resonance imaging showed a sellar mass, and the patient underwent transcranial surgery. Histopathology revealed autoimmune hypophysitis with predominantly CD20 positive B-cell infiltration. Progression of visual field defects necessitated postoperatively anti-inflammatory treatment with prednisolone. Azathioprine was initiated under gradual tapering of prednisolone with stable conditions at first, but relapse followed after dose reduction. Therefore, rituximab treatment was initiated, which resulted in regression of the pituitary mass. Rituximab treatment was discontinued after 25 months. The patient has continuously been in remission for 4 years after rituximab treatment was stopped.ConclusionThis case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis.
Highlights
Autoimmune hypophysitis is a rare condition that often results in enlargement of the pituitary gland and hypopituitarism due to inflammatory infiltration
This case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis
Hypophysitis is a rare condition characterized by inflammatory infiltration of the pituitary gland
Summary
Autoimmune hypophysitis is a rare condition that often results in enlargement of the pituitary gland and hypopituitarism due to inflammatory infiltration. Conclusion: This case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis. Steroid-sparing agents such as azathioprine (an anti-metabolic agent with immunosuppressive activity) are considered in the case of relapse with mass-related symptoms or increased mass size on MRI, need of long-term anti-inflammatory treatment, or intolerable adverse effects to glucocorticoids. Because of progression of visual field defects, treatment with glucocorticoid pulse therapy was initiated: methylprednisolone 500 mg infusion for 3 days, followed by reduced prednisolone doses, that is, 37.5 mg per day.
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