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Related Experiment Video

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Induction of Ocular Surface Inflammation and Collection of Involved Tissues
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IgG4-associated orbital and ocular inflammation.

Cecilia S Lee1, George J Harocopos2, Courtney L Kraus3

  • 1Department 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.

Journal of Ophthalmic Inflammation and Infection
|June 3, 2015
PubMed
Summary

Immunoglobulin G4-related disease (IgG4-RD) can cause orbital and ocular inflammation. Histopathologic evaluation is crucial for diagnosing this condition and guiding targeted therapy.

Keywords:
HistopathologyIgG4-associated ocular inflammationIgG4-associated orbital inflammation

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Area of Science:

  • Ophthalmology
  • Rheumatology
  • Pathology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is an emerging entity causing sclerosing inflammation with IgG4-positive plasma cell infiltration.
  • Previously idiopathic inflammatory syndromes are increasingly reclassified as IgG4-RD based on histopathology.
  • IgG4-RD can affect virtually any organ system, presenting as chronic, relapsing, sclerosing inflammation.

Purpose of the Study:

  • To highlight the diverse manifestations of IgG4-associated orbital and ocular inflammation.
  • To emphasize the critical role of histopathologic evaluation in diagnosing IgG4-RD.
  • To inform ophthalmologists about IgG4-RD as a differential diagnosis for chronic sclerosing inflammation.

Main Methods:

  • Case series reporting three distinct presentations of IgG4-associated ocular and orbital inflammation.
  • Detailed histopathologic evaluation, including immunohistochemistry, was performed on biopsy specimens.
  • Clinical correlation of histopathologic findings with patient presentations.

Main Results:

  • Case 1: Recurrent, treatment-refractory sclero-uveitis with an IgG4-related component, diagnosed as granulomatosis with polyangiitis.
  • Case 2: Pachymeningitis with optic neuritis leading to permanent visual loss.
  • Case 3: Orbital inflammatory pseudotumor.
  • All cases required thorough histopathologic evaluation for accurate diagnosis.

Conclusions:

  • IgG4-associated disease presents with a wide spectrum of ocular and orbital manifestations.
  • Ophthalmologists should consider IgG4-RD in cases of chronic, sclerofibrosing inflammation, especially with multi-system involvement.
  • Accurate diagnosis via histology is essential for timely and targeted therapeutic interventions, potentially reducing unnecessary diagnostic procedures.