Survey of Ophthalmology
Volume 57, Issue 1 , Pages 26-33, 2 January 2012

IgG4-Related Systemic Disease as a Cause of “Idiopathic” Orbital Inflammation, Including Orbital Myositis, and Trigeminal Nerve Involvement

  • Zachary S. Wallace, MD

      Affiliations

    • Department of Medicine, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • ,
  • Arezou Khosroshahi, MD

      Affiliations

    • Department of Medicine, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • Division of Rheumatology, Allergy, & Immunology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA
    • ,
  • Frederick A. Jakobiec, MD

      Affiliations

    • Department of Pathology, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • The Cogan Eye Pathology Laboratory, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA
    • ,
  • Vikram Deshpande, MD

      Affiliations

    • Department of Pathology, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • ,
  • Mark P. Hatton, MD

      Affiliations

    • Department of Ophthalmology, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • ,
  • Jill Ritter, MD

      Affiliations

    • Private practice, New Jersey, USA
    • ,
  • Judith A. Ferry, MD

      Affiliations

    • Department of Pathology, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • ,
  • John H. Stone, MD, MPH

      Affiliations

    • Department of Medicine, Harvard Medical School, Cambridge, Massachusetts, and the Massachusetts General Hospital, Boston, Massachusetts, USA
    • Division of Rheumatology, Allergy, & Immunology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA
    • Corresponding Author InformationReprint address: Dr. John H. Stone, Rheumatology Unit / Yawkey 2, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114.

published online 24 October 2011.

Stefan Seregard and Milton Boniuk, Editors

Abstract 

IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.

Key words: dacryoadenitis, Idiopathic Inflammation, IgG4-related systemic disease, myositis, orbital pseudotumor, rituximab

 

PII: S0039-6257(11)00165-2

doi:10.1016/j.survophthal.2011.07.004

Survey of Ophthalmology
Volume 57, Issue 1 , Pages 26-33, 2 January 2012

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