Abstract
IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.
Keywords:
Bilateral IgG4-related orbital disease; peripheral ulcerative keratitis; sclerosing autoimmune pancreatitis; steroid and rituximab treatment.
MeSH terms
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Adult
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Autoimmune Diseases / diagnosis
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Autoimmune Diseases / drug therapy
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Corneal Ulcer / diagnosis*
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Corneal Ulcer / drug therapy
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Drug Combinations
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Follow-Up Studies
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Glucocorticoids / therapeutic use*
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Humans
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Immunoglobulin G / blood*
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Immunologic Factors / therapeutic use
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Male
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Orbital Diseases / diagnosis*
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Orbital Diseases / drug therapy
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Pancreatitis / diagnosis*
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Pancreatitis / drug therapy
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Paraproteinemias / diagnosis*
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Paraproteinemias / drug therapy
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Plasma Cells / pathology
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Rituximab / therapeutic use*
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Tomography, X-Ray Computed
Substances
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Drug Combinations
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Glucocorticoids
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Immunoglobulin G
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Immunologic Factors
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Rituximab