IgG4-related ophthalmic disease

IgG4-related ophthalmic disease

Right optic disc lesion in IgG4-ROD

Mass lesion around the right optic disc in a 44-year-old man with IgG4-related ophthalmic disease and a serum IgG4 of 599 mg/dL.[1] (T2-weighted MRI)
Classification and external resources
Specialty Ophthalmology

IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital manifestations of the systemic condition IgG4-related disease,[2] which is characterised by lymphoplasmacytic infiltration, follicle formation and fibrosclerosis of involved structures. It can involve one or more of the orbital structures.

Frequently involved structures include the lacrimal glands, extraocular muscles, infraorbital nerve, supraorbital nerve and eyelids.[3][4][5][6][7][8]

A prompt response to steroid therapy is a characteristic feature of IgG4-ROD in most cases, as it is with other manifestations of IgG4-related disease, unless significant fibrosis has already occurred.[2][3]

Nomenclature

Although IgG4-related ophthalmic disease is the recommended name for all orbital manifestations of IgG4-related disease, more precise terminology for the various anatomical variations of the condition can be used. This includes:[2][3]

Names previously used in the diagnosis of cases now considered to be IgG4-ROD have included idiopathic orbital inflammatory disease and orbital pseudotumor.[2]

Symptoms

Breakdown of diagnosis in orbital lymphoproliferative disorders in a Japanese study[1]

Symptoms, if any, can be mild even in the presence of significant swelling or masses.

Lacrimal gland involvement may cause swelling of the upper eyelid, or proptosis if there is severe swelling. Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, or proptosis.[3]

IgG4-related ophthalmic disease has been estimated to account for approximately 25% of all cases of proptosis, eyelid swelling and other features of orbital swelling.[1][3]

Imaging

The extent of inflammation that can occur in IgG4-ROD is well demonstrated on magnetic resonance imaging (MRI):

Enlargements in the left inferior rectus muscle and infraorbital nerve (arrow) in a 65-year-old man with a serum IgG4 of 404 mg/dL.[1] (T2-weighted MRI)
Swelling of the left superior and lateral rectus muscles, a mass lesion around the left optic disc (arrow), and enlargements of the left supraorbital nerve and the right infraorbital nerve (arrow heads) in a 60-year-old man with a serum IgG4 of 463 mg/dL.[1] (T1-weighted MRI)
Bilateral supraorbital nerve enlargements (arrows) and right infraorbital nerve (arrow head) enlargement in a 47-year-old woman with a serum IgG4 of 1000 mg/dL.[1] (T1-weighted MRI)

References

  1. 1 2 3 4 5 6 Masayuki Takahira; Yoshiaki Ozawa; Mitsuhiro Kawano; Yoh Zen; Shoko Hamaoka; Kazunori Yamada; Kazuhisa Sugiyama (2012). "Clinical Aspects of IgG4-Related Orbital Inflammation in a Case Series of Ocular Adnexal Lymphoproliferative Disorders". International Journal of Rheumatology 2012. doi:10.1155/2012/635473. PMC 3323851. PMID 22548072. 635473.
  2. 1 2 3 4 John H. Stone; Arezou Khosroshahi; Vikram Deshpande; John K. C. Chan; J. Godfrey Heathcote; Rob Aalberse; Atsushi Azumi; Donald B. Bloch; William R. Brugge; Mollie N. Carruthers; Wah Cheuk; Lynn Cornell; Carlos Fernandez-Del Castillo; Judith A. Ferry; David Forcione; Günter Klöppe; Daniel L. Hamilos; Terumi Kamisawa; Satomi Kasashima; Shigeyuki Kawa; Mitsuhiro Kawano; Yasufumi Masaki; Kenji Notohara; Kazuichi Okazaki; Ji Kon Ryu; Takako Saeki; Dushyant Sahani; Yasuharu Sato; Thomas Smyrk; James R. Stone; Masayuki Takahira; Hisanori Umehara; George Webster; Motohisa Yamamoto; Eunhee Yi; Tadashi Yoshino; Giuseppe Zamboni; Yoh Zen; Suresh Chari (October 2012). "Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations". Arthritis & Rheumatism 64 (10): 3061–3067. doi:10.1002/art.34593. PMID 22736240.
  3. 1 2 3 4 5 Takahira, Masayuki; Azumi, Atsushi (21 November 2013). "Chapter 12: Ophthalmology". In Hisanori Umehara; Kazuichi Okazaki; John H. Stone; Shigeyuki Kawa; Mitsuhiro Kawano. IgG4-Related Disease. Springer Science & Business Media. pp. 77–84. doi:10.1007/978-4-431-54228-5. ISBN 978-4-431-54227-8.
  4. ↑ José Antonio Plaza; James A. Garrity; Ahmet Dogan; Anuradha Ananthamurthy; Thomas E. Witzig; Diva R. Salomão (11 April 2011). "Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease". Archives of Ophthalmology (American Medical Association) 129 (4): 421–428. doi:10.1001/archophthalmol.2011.16. PMID 21482868.
  5. ↑ Thomas G. Hardy; Alan A. McNab; Geoffrey E. Rose (June 2014). "Enlargement of the infraorbital nerve: an important sign associated with orbital reactive lymphoid hyperplasia or immunoglobulin g4-related disease". Ophthalmology (American Academy of Ophthalmology) 121 (6): 1297–1303. doi:10.1016/j.ophtha.2013.12.028. PMID 24613826.
  6. ↑ Anuradha Jayaprakasam; Dominic O'Donovan; Cornelius Rene (May 2014). "Infraorbital nerve enlargement due to IgG4-related disease". Eye (London: Royal College of Ophthalmologists) 28 (5): 628–629. doi:10.1038/eye.2014.32. PMC 4017115. PMID 24577252.
  7. ↑ Dai Inoue; Yoh Zen; Yasuharu Sato; Hitoshi Abo; Hiroshi Demachi; Akio Uchiyama; Toshifumi Gabata; Osamu Matsui (2012). "IgG4-Related Perineural Disease". International Journal of Rheumatology 2012. doi:10.1155/2012/401890. PMC 3317227. PMID 22523496. 401890.
  8. ↑ Tiina Leivo; Sari Koskenmies; Marita Uusitalo; Olli Tynninen (August 2015). "IgG4-related disease mimicking chalazion in the upper eyelid with skin manifestations on the trunk". International Ophthalmology 35 (4): 595–597. doi:10.1007/s10792-015-0070-x. PMID 25834990.
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