Recurrent autoimmune hypophysitis treated with rituximab: a case report

• Published in: Journal of medical case reports Vol. 15; no. 1; p. 591
• Main Authors: Kruse, Maria, Olesen, Thomas Bastholm, Markovic, Ljubo, Glintborg, Dorte, Andersen, Marianne Skovsager
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 15.12.2021; BioMed Central; BMC
• Subjects: Adult; Anti-Inflammatory Agents - therapeutic use; Autoimmune hypophysitis; Autoimmune Hypophysitis - drug therapy; Azathioprine; B cells; Care and treatment; Case Report; Case reports; CD20+ B-cells; Corticosteroids; Diabetes; Drug dosages; Edema; Growth hormones; Headaches; Histochemistry; Humans; Immunotherapy; Inflammation; Lymphocytic hypophysitis; Lymphoma; Magnetic Resonance Imaging; Male; Monoclonal antibodies; Monoclonal antibody; Patients; Pituitary Diseases - drug therapy; Pituitary gland; Prednisolone; Prednisolone - therapeutic use; Primary hypophysitis; Recurrence; Rituximab - therapeutic use; Steroids; Tumors; Weight loss; Autoimmune hypophysitis; CD20+ B-cells; Immunotherapy; Lymphocytic hypophysitis; Rituximab; Monoclonal antibody; Primary hypophysitis
• ISSN: 1752-1947; 1752-1947
• DOI: 10.1186/s13256-021-03146-0

Autoimmune 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. We 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. This case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis.