title: IgG4-associated orbital and ocular inflammation.
abstract: BACKGROUND
  IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation.
  
  
  RESULTS
  We report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry).
  
  
  CONCLUSIONS
  IgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy.
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA ; Department of Ophthalmology, University of Washington, Seattle, WA USA.
authors.forename: Cecilia S
authors.initials: CS
authors.lastname: Lee
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA ; Department of Pathology and Immunology, Washington University in St. Louis, St. Louis, MO USA.
authors.forename: George J
authors.initials: GJ
authors.lastname: Harocopos
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA ; Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD USA.
authors.forename: Courtney L
authors.initials: CL
authors.lastname: Kraus
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA ; Department of Ophthalmology, University of British Columbia, Vancouver, BC Canada.
authors.forename: Aaron Y
authors.initials: AY
authors.lastname: Lee
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA.
authors.forename: Gregory P
authors.initials: GP
authors.lastname: Van Stavern
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Couch
authors.affiliation: Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, CB 8096, 660 S. Euclid, St. Louis, MO 63110 USA.
authors.forename: P Kumar
authors.initials: PK
authors.lastname: Rao
keywords: Histopathology; IgG4-associated ocular inflammation; IgG4-associated orbital inflammation