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A case series of patients with isolated IgG4-related hypophysitis treated with rituximab

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Title: A case series of patients with isolated IgG4-related hypophysitis treated with rituximab
Authors: Boharoon, H
Tomlinson, J
Limback-Stanic, C
Gontsarova, A
Martin, N
Hatfield, E
Meeran, M
Nair, R
Mendoza, N
Levy, J
McAdoo, S
Pusey, C
Wernig, F
Item Type: Journal Article
Abstract: Context 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. Case description We report three female patients with MRI findings suggestive of hypophysitis. All patients underwent transsphenoidal biopsy and fulfilled diagnostic criteria for IgG4-related hypophysitis. Treatment with GCs (GC) 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 dose reduction. All three patients received RTX and Patients 2 and 3 received further courses when symptoms returned and B-cells repopulated. Patient 3 did not receive RTX until 12 months from onset of symptoms. Patient 1 was not able to have further RTX treatments due to an allergic reaction when receiving the second dose. RTX treatment resulted in sustained remission and full recovery of anterior pituitary function in Patients 1 and 2 with complete resolution of pituitary enlargement. By contrast, Patient 3 only showed symptomatic response following RTX treatment, but pituitary enlargement and hypofunction persisted. Conclusion RTX treatment for IgG4-related hypophysitis resulted in sustained remission in two patients treated early in the disease process, but only achieved partial response in a patient with chronic disease suggesting that early therapeutic intervention may be crucial to avoid irreversible changes.
Issue Date: 21-Apr-2021
Date of Acceptance: 17-Apr-2020
URI: http://hdl.handle.net/10044/1/79529
DOI: 10.1210/jendso/bvaa048
ISSN: 2472-1972
Publisher: Endocrine Society
Start Page: 1
End Page: 9
Journal / Book Title: Journal of the Endocrine Society
Volume: 4
Issue: 6
Copyright Statement: © Endocrine Society 2020. js.2020-00091 See endocrine.org/publications for Accepted Manuscript disclaimer and additional information. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-ncnd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
Publication Status: Published
Online Publication Date: 2021-04-21
Appears in Collections:Department of Immunology and Inflammation
Department of Metabolism, Digestion and Reproduction
Faculty of Medicine