Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2015 Oct-Dec;22(4):447-51.
doi: 10.4103/0974-9233.167807.

Sclerosing Lesions of the Orbit: A Review

Gautam Lokdarshi  1 , Neelam Pushker  1 , Mandeep S Bajaj  1
Affiliations
Review

Sclerosing Lesions of the Orbit: A Review

Gautam Lokdarshi et al. Middle East Afr J Ophthalmol. 2015 Oct-Dec.

Abstract

Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome) and neoplasms (lymphoma, metastatic breast carcinoma) should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis.

Keywords: IgG4; Inflammation; Neoplasm; Orbit; Sclerosing.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Bilateral proptosis with frozen globe (complete restriction of ocular movements) in a middle aged female with IgG4-related orbitopathy
Figure 2
Figure 2
Computed tomography (axial section) of the same patient in Figure 1 showing bilateral diffuse infiltrating lesion of the orbits, sparing bone
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

  • Idiopathic Orbital Inflammation: Review of Literature and New Advances.
    Yeşiltaş YS, Gündüz AK. Yeşiltaş YS, et al. Middle East Afr J Ophthalmol. 2018 Apr-Jun;25(2):71-80. doi: 10.4103/meajo.MEAJO_44_18. Middle East Afr J Ophthalmol. 2018. PMID: 30122852 Free PMC article. Review.
  • Rare Diseases of the Orbit.
    Kisser U, Heichel J, Glien A. Kisser U, et al. Laryngorhinootologie. 2021 Apr;100(S 01):S1-S79. doi: 10.1055/a-1384-4641. Epub 2021 Apr 30. Laryngorhinootologie. 2021. PMID: 34352903 Free PMC article. Review.
  • CT and MR imaging of orbital inflammation.
    Ferreira TA, Saraiva P, Genders SW, Buchem MV, Luyten GPM, Beenakker JW. Ferreira TA, et al. Neuroradiology. 2018 Dec;60(12):1253-1266. doi: 10.1007/s00234-018-2103-4. Epub 2018 Oct 11. Neuroradiology. 2018. PMID: 30310941 Free PMC article. Review.
  • IgG4-Related Orbitopathy.
    Chaudhry IA. Chaudhry IA. Middle East Afr J Ophthalmol. 2015 Oct-Dec;22(4):405-6. doi: 10.4103/0974-9233.167816. Middle East Afr J Ophthalmol. 2015. PMID: 26692707 Free PMC article. No abstract available.
  • Case of IgG4 orbitopathy's remarkable response to oral corticosteroid therapy.
    Balakrishnan N, Meel R, Das D. Balakrishnan N, et al. BMJ Case Rep. 2020 Aug 26;13(8):e236442. doi: 10.1136/bcr-2020-236442. BMJ Case Rep. 2020. PMID: 32847889 Free PMC article. No abstract available.

References

    1. Rootman J. 2nd ed. Vol. 156. USA: Lippincott Williams and Wilkins; 2003. Diseases of the Orbit: A Multidisciplinary Approach; pp. 455–506.
    1. Thorne JE, Volpe NJ, Wulc AE, Galetta SL. Caught by a masquerade: Sclerosing orbital inflammation. Surv Ophthalmol. 2002;47:50–4. - PubMed
    1. Liu CH, Ma L, Ku WJ, Kao LY, Tsai YJ. Bilateral idiopathic sclerosing inflammation of the orbit: Report of three cases. Chang Gung Med J. 2004;27:758–65. - PubMed
    1. Abramovitz JN, Kasdon DL, Sutula F, Post KD, Chong FK. Sclerosing orbital pseudotumor. Neurosurgery. 1983;12:463–8. - PubMed
    1. Gordon LK. Orbital inflammatory disease: A diagnostic and therapeutic challenge. Eye (Lond) 2006;20:1196–206. - PubMed