Rituximab for corticosteroid-resistant relapsing IgG4-related ophthalmic disease: A case report and literature review

Authors

  • Dr. Sadra Habibi Moini Iranian Center of Neurological Research, Neuroscience Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author
  • Dr. Samaneh Kabiri Department of Neurology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author
  • Prof. Mohammad Hossein Harirchian Iranian Center of Neurological Research, Neuroscience Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author
  • Prof. Ehsan Mohammadianinejad Iranian Center of Neurological Research, Neuroscience Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author
  • Dr. Arezoo Behbood Department of Neurology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author
  • Dr. Rahele Sefidian Department of Neurology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Author

Keywords:

Rituximab, IgG4-related ophthalmic disease, orbital pseudotumor

Abstract

Abstract

Background IgG4- related ophthalmic disease (IgG4-ROD) is a newly recognized term for IgG-related disease (IgG4-RD) patients with ocular adnexal involvement. This study aims to present a case of bilateral IgG4-ROD to highlight its diagnostic approaches and therapeutic options

Case presentation We report a case of 34-year-old woman presenting with bilateral proptosis. Imaging revealed involvement of lacrimal glands as well as a right retrobulbar mass encasing ocular muscles. Despite having a normal serum IgG4 level (28 mg/dl), the diagnosis of IgG4-ROD was made based on histopathologic and radiologic findings. Remission was attained with rituximab (RTX) after a recurrence of symptoms while being on glucocorticoids (GC) and azathioprine (AZA).

Conclusions IgG4-ROD can manifest as a pseudotumor with involvement of adjacent tissues and should be considered in approaching ocular lesions. Appropriate work-up to detect multi-organ involvement is essential. Refractory or recurrent IgG4-ROD is not unusual and therefore maintenance or intensifying treatment can prevent irreversible organ damage.

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Published

22-10-2024

Issue

Vol. 1 (2024): Caspian Journal of Case Reports

Section

Case Reports

License

Copyright (c) 2024 Caspian Journal of Case Reports

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How to Cite

Habibi Moini, S. (2024) “Rituximab for corticosteroid-resistant relapsing IgG4-related ophthalmic disease: A case report and literature review”, Caspian Journal of Case Reports, 1, pp. 9–22. Available at: https://caspian-jcr.com/index.php/cjcr/article/view/6 (Accessed: 3 April 2025).