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.
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Authors | 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 |
Journal | Ophthalmic plastic and reconstructive surgery
(Ophthalmic Plast Reconstr Surg)
2014 Sep-Oct
Vol. 30
Issue 5
Pg. e122-5
ISSN: 1537-2677 [Electronic] United States |
PMID | 24481505
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Antibodies, Monoclonal, Murine-Derived
- Antigens, CD20
- Immunoglobulin G
- Immunologic Factors
- Rituximab
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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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