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.

References 

  1. Ahmed M, Niffenegger JH, Jakobiec FA, et al. Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation. Int Ophthalmol. 2008;28:35–46
  2. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303–332
  3. Costa RM, Dumitrascu OM, Gordon LK. Orbital myositis: diagnosis and management. Curr Allergy Asthma Rep. 2009;9:316–323
  4. Espinoza GM. Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep. 2010;12:443–447
  5. Faller M, Purohit A, Kennel N, et al. Systemic sarcoidosis initially presenting as an orbital tumour. Eur Respir J. 1995;8:474–476
  6. Geyer JT, Deshpande V. IgG4-associated sialadenitis. Curr Opin Rheumatol. 2011;23:95–101
  7. Golnik KC. Neuro-ophthalmologic manifestations of systemic disease: rheumatologic/Inflammatory. Ophthalmol Clin North Am. 2004;17:389–396vi
  8. Jakobiec FA, Stacy RC, Mehta M, et al. IgG4-positive dacryoadenitis and Kuttner submandibular sclerosing inflammatory tumor. Arch Ophthalmol. 2010;128:942–944
  9. Katsura M, Morita A, Horiuchi H, et al. IgG4-Related inflammatory pseudotumor of the trigeminal nerve: another component of IgG4-related sclerosing disease?. AJNR Am J Neuroradiol. 2010;Epub ahead of print
  10. Khosroshahi A, Stone JH. IgG4-related systemic disease: the age of discovery. Curr Opin Rheumatol. 2011;23:72–73
  11. Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum. 2010;62:1755–1762
  12. Khosroshahi A, Stone JR, Pratt DS, et al. Painless jaundice with serial multi-organ dysfunction. Lancet. 2009;373:1494
  13. Kubota T, Moritani S, Katayama M, et al. Ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder. Arch Ophthalmol. 2010;128:577–584
  14. Lutt JR, Lim LL, Phal PM, et al. Orbital inflammatory disease. Semin Arthritis Rheum. 2008;37:207–222
  15. Mavrikakis I, Rootman J. Diverse clinical presentations of orbital sarcoid. Am J Ophthalmol. 2007;144:769–775
  16. McKelvie PA. Ocular adnexal lymphomas: a review. Adv Anat Pathol. 2010;17:251–261
  17. Rueda JC, Duarte-Rey C, Casas N. Successful treatment of relapsing autoimmune pancreatitis in primary Sjogren's syndrome with rituximab: report of a case and review of the literature. Rheumatol Int. 2009;29:1481–1485
  18. Sato Y, Ohshima K, Ichimura K, et al. Ocular adnexal IgG4-related disease has uniform clinicopathology. Pathol Int. 2008;58:465–470
  19. Schoser BG. Ocular myositis: diagnostic assessment, differential diagnoses, and therapy of a rare muscle disease—five new cases and review. Clin Ophthalmol. 2007;1:37–42
  20. Shinder R, Al-Zubidi N, Esmaeli B. Survey of orbital tumors at a comprehensive cancer center in the United States. Head Neck. 2011;33(5):610–614
  21. Smyrk TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol. 2011;23:74–79
  22. Stacy RC, Jakobiec FA, Schoenfield L, et al. Unifocal and multifocal reactive lymphoid hyperplasia vs follicular lymphoma of the ocular adnexa. Am J Ophthalmol. 2010;150:412–426.e1
  23. Tarabishy AB, Schulte M, Papaliodis GN, et al. Wegener's granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Surv Ophthalmol. 2010;55:429–444
  24. Topazian M, Witzig TE, Smyrk TC, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2008;6:364–366

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

Article Tools

* Image Usage & Resolution