Immunoglobulin G4-posltive Sclerosing Idiopathic Orbital Inflammation: New Neuro-ophthalmological Presentations

摘要

We report two rare cases of biopsy proven Immunoglobulin G4-related sclerosing orbital inflammation (IgG4SOI). The first case had intracranial involvement which, to our knowledge, is the first IgG4SOI case with serum cerebrospinal fluid abnormalities and the second case had an unusual presentation of a compressive optic neuropathy and systemic lymphadenopathy. Immunoglobulin G4 (IgG4) disease in the orbit or IgG4-related sclerosing orbital inflammation (IgG4SOI) is a rare condition with a variety of clinical presentations that may be associated with systemic disease. It is an inflammatory disease likely of autoimmune origin, characterised by either high levels of serum IgG4 or pathologically by predominant infiltration of plasmacytic cells with IgG4 expression.