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

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. 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.
AuthorsTony 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
JournalOphthalmic plastic and reconstructive surgery (Ophthalmic Plast Reconstr Surg) 2014 Sep-Oct Vol. 30 Issue 5 Pg. e122-5 ISSN: 1537-2677 [Electronic] United States
PMID24481505 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Antibodies, Monoclonal, Murine-Derived
  • Antigens, CD20
  • Immunoglobulin G
  • Immunologic Factors
  • Rituximab
Topics
  • Aged
  • Antibodies, Monoclonal, Murine-Derived (therapeutic use)
  • Antigens, CD20
  • Blindness (etiology, therapy)
  • Combined Modality Therapy
  • Decompression, Surgical
  • Humans
  • Immunoglobulin G (blood)
  • Immunologic Factors (therapeutic use)
  • Magnetic Resonance Imaging
  • Male
  • Nerve Compression Syndromes (diagnosis, etiology, therapy)
  • Optic Nerve Diseases (diagnosis, etiology, therapy)
  • Orbital Myositis (complications, diagnosis)
  • Orbital Pseudotumor (complications, diagnosis)
  • Rituximab
  • Tomography, X-Ray Computed

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