IgG4-associated orbital and ocular inflammation

Cecilia S. Lee, George J. Harocopos, Courtney L. Kraus, Aaron Y. Lee, Gregory P. Van Stavern, Steven M. Couch, P. Kumar Rao

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44 Scopus citations


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. Findings: 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.

Original languageEnglish
Article number15
JournalJournal of Ophthalmic Inflammation and Infection
Issue number1
StatePublished - Dec 1 2015


  • Histopathology
  • IgG4-associated ocular inflammation
  • IgG4-associated orbital inflammation


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