Successful "medical" orbital decompression with adjunctive rituximab for severe visual loss in IgG4-related orbital inflammatory disease with orbital myositis.
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.
*Ocular Plastics Unit, Department of Ophthalmology, and †Medical Imaging Department, Prince of Wales Hospital; ‡Medical Imaging Department, Sydney Children's Hospital, Sydney, Australia; §Department of Histopathology, St Vincent's Hospital Melbourne, Melbourne, Australia; ║Department of Radiation Oncology, Prince of Wales Hospital, Sydney, Australia; and ¶Prince of Wales Clinical School, University of New South Wales, Sydney, Australia.
Tony Shang-Chuan
TS
Chen
Edwin
E
Figueira
Oliver C F
OC
Lau
Penny A
PA
McKelvie
Robert I
RI
Smee
Laughlin C
LC
Dawes
Ashish
A
Agar
Geoff
G
Wilcsek
Ian C
IC
Francis
D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018951:Antigens, CD20; D001766:Blindness / Q000209:etiology / Q000628:therapy*; D003131:Combined Modality Therapy; D019299:Decompression, Surgical; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D009408:Nerve Compression Syndromes / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D055622:Orbital Myositis / Q000150:complications* / Q000175:diagnosis; D016727:Orbital Pseudotumor / Q000150:complications* / Q000175:diagnosis; D000069283:Rituximab; D014057:Tomography, X-Ray Computed
D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab