Successful "Medical" orbital decompression with adjunctive rituximab for severe visual loss in IgG4-related orbital inflammatory disease with orbital myositis

Tony Shang Chuan Chen, Edwin Figueira, Oliver C F Lau, Penny A. McKelvie, Robert I. Smee, Laughlin C. Dawes, Ashish Agar, Geoff Wilcsek, Ian C. Francis*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

IgG4-related disease (IgG4-RD) is an inflammatory condition of unknown etiology that can cause tumefactive lesions in a number of tissues and organs, including the orbit and ocular adnexa. Diagnostic criteria for IgG4-RD, including pathology and clinical features and pathology, have been recently proposed.1 This study presents the first case of unilateral acute visual loss secondary to IgG4-related orbital inflammatory disease with orbital myositis that was complicated by severe compressive optic neuropathy. After initial treatment with pulsed intravenous methylprednisolone, followed by rituximab and radiotherapy, there was a marked improvement in orbital inflammation and clinical and radiological improvement in the compressive optic neuropathy. After 9 months of follow up, the orbital inflammatory disease remained in remission.

Original languageEnglish
Pages (from-to)e122-e125
Number of pages4
JournalOphthalmic plastic and reconstructive surgery
Volume30
Issue number5
DOIs
Publication statusPublished - 1 Feb 2014
Externally publishedYes

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