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 Table of Contents  
Year : 2020  |  Volume : 8  |  Issue : 1  |  Page : 159-160

Radiological signs on orbital imaging of immunoglobulin G4-related disease

Department of Ophthalmology, Tung Wah Eastern Hospital, HKSAR, Hong Kong

Date of Submission07-Oct-2019
Date of Decision08-Dec-2019
Date of Acceptance10-Dec-2019
Date of Web Publication20-Jun-2020

Correspondence Address:
Dr. Sunny Chi Lik Au
9/F, MO Office, Lo Ka Chow Memorial Ophthalmic Centre, Tung Wah Eastern Hospital, 19 Eastern Hospital Road, Causeway Bay, HKSAR
Hong Kong
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/amhs.amhs_142_19

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Immunoglobulin G4 (IgG4)-related disease has strong organ predilections, and orbital involvement is not rare. Eyelid puffiness with erythema, extraocular movement deficit with proptosis are common presentations, which could sometimes mimic thyroid-associated orbitopathy. With a better understanding on orbital radiology, these “hot eyes” could be managed better.

Keywords: Computed tomography, immunoglobulin G4-related disease, proptosis

How to cite this article:
Lik Au SC, Chan E. Radiological signs on orbital imaging of immunoglobulin G4-related disease. Arch Med Health Sci 2020;8:159-60

How to cite this URL:
Lik Au SC, Chan E. Radiological signs on orbital imaging of immunoglobulin G4-related disease. Arch Med Health Sci [serial online] 2020 [cited 2022 Nov 26];8:159-60. Available from: https://www.amhsjournal.org/text.asp?2020/8/1/159/287348

A 45-year-old female presented with bilateral puffy eyelid swelling and proptosis. She had a recent history of salivary gland swelling and cervical lymphadenopathy, with submandibular gland biopsy showing dense lymphoplasmacytic infiltrate rich in plasma cells. Immunostaining showed IgG4/IgG ratio >40%, with IgG4+ plasma cell count of 80–100/high-power field. Serum IgG4 level was elevated 4.456 g/L (laboratory reference range: 0.168–1.000). Orbital computed tomography revealed proptosis, lacrimal glands, and orbital soft tissue swelling. Extraocular muscles were swollen without tendon sparing, and bilateral infraorbital nerves were enlarged together with the bony infraorbital canal [Figure 1]. Biopsy of the lacrimal gland revealed similar immunohistopathological findings as above and confirmed the diagnosis of IgG4-related disease (IgG4-RD).
Figure 1: Coronal cut of computed tomography of orbit showing bilateral enlarged infraorbital nerve and bony canal. Left proptosis is evidenced by the relatively anterior position of the left globe resulted from orbital soft tissue swelling

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Proptosis could be unilateral arising from localized pathology or bilateral due to systemic inflammatory diseases. Unilateral pseudoproptosis could occur when there is enophthalmos over the contralateral eye, such as in orbital blowout fracture or phthisis bulbi [Figure 2]. Common etiology of unilateral proptosis includes orbital cellulitis, elongated eyeball in high myopia, and space-occupying lesion within or of the bony orbit. In contrast, bilateral proptosis is commonly seen in thyroid-associated orbitopathy (TAO), IgG4-related ophthalmic disease, or idiopathic orbital inflammatory disease. TAO usually gives the characteristic of swelling of extraocular muscle belly with tendon sparing,[1] which differentiates it from IgG4-RD. Lagophthalmos must be addressed in proptotic eye, to prevent corneal exposure keratopathy.
Figure 2: Another immunoglobulin G4-related disease patient, with a history of childhood left eye trauma resulting in phthisis bulbi, got right eye pseudoproptosis progressing to genuine proptosis. Hyperdensity over the left phthisical eye highlights the calcification inside

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Infraorbital nerve enlargement is an uncommon radiological sign and strongly relates to IgG4-RD.[2] Histologically, mainly the epineurium was involved by the inflammatory process, whereas perineurium and nerve fascicules were spared.[3] The presence of infraorbital nerve enlargement correlates with proptosis, diffuse, and inferiorly located orbital inflammation, with a higher rate of steroid dependency and recurrence.[4] However, associated medical history is important, as enlargement of infraorbital canal could also be seen after maxillary sinus surgery.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Maheshwari R, Weis E. Thyroid associated orbitopathy. Indian J Ophthalmol 2012;60:87-93.  Back to cited text no. 1
[PUBMED]  [Full text]  
Hardy TG, McNab AA, Rose GE. Enlargement of the infraorbital nerve: An important sign associated with orbital reactive lymphoid hyperplasia or immunoglobulin g4-related disease. Ophthalmology 2014;121:1297-303.  Back to cited text no. 2
Inoue D, Zen Y, Sato Y, Abo H, Demachi H, Uchiyama A, et al. IgG4-Related perineural disease. Int J Rheumatol 2012;2012:401890.  Back to cited text no. 3
Lee KH, Han SH, Yoon JS. Implications of enlarged infraorbital nerve in idiopathic orbital inflammatory disease. Br J Ophthalmol 2016;100:1295-300.  Back to cited text no. 4
Fukui H, Kashiwagi N, Murakami T, Watanabe Y, Hyodo T, Ishi K, et al. Enlargement of the infraorbital canal following Caldwell-Luc surgery. Jpn J Radiol 2017;35:532-8.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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