Abstract
IgG4 is a newly known systemic disorder. It can present as orbital inflammation or as an orbital mass. We describe the case of a 5-year-old child with proptosis and globe dystopia who went on to have an orbital biopsy. Initially this was reported to be idiopathic orbital inflammation. After IgG4-RD was considered in the differential, the orbital biopsy was revisited, immunohistochemistry was done and the patient's serum was tested for IgG subclass levels. IgG4-RD was diagnosed and the patient was subsequently treated with prednisone and mycophenolate and made a good recovery. This is the youngest reported case of IgG4-RD in a child.
MeSH terms
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Child, Preschool
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Diagnosis, Differential
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Drug Therapy, Combination
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Female
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Humans
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Hypergammaglobulinemia / diagnosis*
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Hypergammaglobulinemia / drug therapy
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Hypergammaglobulinemia / immunology
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Immunoglobulin G / blood*
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Immunosuppressive Agents / therapeutic use
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Magnetic Resonance Imaging
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Mycophenolic Acid / analogs & derivatives
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Mycophenolic Acid / therapeutic use
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Orbital Neoplasms / diagnosis*
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Orbital Neoplasms / drug therapy
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Orbital Neoplasms / immunology
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Orbital Pseudotumor / diagnosis*
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Orbital Pseudotumor / drug therapy
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Orbital Pseudotumor / immunology
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Plasma Cells / immunology
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Prednisone / therapeutic use
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Tomography, X-Ray Computed
Substances
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Immunoglobulin G
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Immunosuppressive Agents
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Mycophenolic Acid
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Prednisone