A Case Series of Patients with Isolated IgG4-related Hypophysitis Treated with Rituximab

Journal of the Endocrine Society
Hessa BoharoonFlorian Wernig

Abstract

The acute presentation of immunoglobulin G4 (IgG4)-related hypophysitis can be indistinguishable from other forms of acute hypophysitis, and histology remains the diagnostic gold standard. The high recurrence rate necessitates long-term immunosuppressive therapy. Rituximab (RTX) has been shown to be effective in systemic IgG4-related disease (IgG4-RD), but experience with isolated pituitary involvement remains limited. We report 3 female patients with MRI findings suggestive of hypophysitis. All patients underwent transsphenoidal biopsy and fulfilled diagnostic criteria for IgG4-related hypophysitis. Treatment with glucocorticoids (GCs) resulted in good therapeutic response in Patients 1 and 2, but the disease recurred on tapering doses of GCs. GC treatment led to emotional lability in Patient 3, necessitating a dose reduction. All 3 patients received RTX and Patients 2 and 3 received further courses of treatment when symptoms returned and B-cells repopulated. Patient 3 did not receive RTX until 12 months from the onset of symptoms. Patient 1 was not able to have further RTX treatments due to an allergic reaction when receiving the second dose. Rituximab treatment resulted in sustained remission and full recovery of anterior pi...Continue Reading

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Citations

Apr 11, 2021·Endocrine·Alireza AmirbaiglooMehdi Zeinalizadeh
Sep 17, 2021·The Journal of Clinical Endocrinology and Metabolism·Fabienne LangloisMaria Fleseriu
Dec 3, 2020·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Josh Waytz, Reem Jan

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Methods Mentioned

BETA
surgical resection
biopsy
biopsies
ESR

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