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ORIGINAL RESEARCH article

Front. Immunol.
Sec. Multiple Sclerosis and Neuroimmunology
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1516909
This article is part of the Research Topic Advances in Ocular Autoimmune Diseases View all articles

Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumorspseudotumours with intracranial extension

Provisionally accepted
Yuyu Li Yuyu Li Mingming Sun Mingming Sun Biyue Chen Biyue Chen Xintong Xu Xintong Xu Yuhang Wang Yuhang Wang Quangang Xu Quangang Xu Huanfen Zhou Huanfen Zhou *Wei Shihui Wei Shihui *
  • Department of Ophthalmology, The Third Medical Center of Chinese PLA General Hospital, Yuzhong, Chongqing, China

The final, formatted version of the article will be published soon.

    Abstract Objective: The aim of this study was to compare the efficacy of low-dose rituximab (RTX) and immunosuppressants in treating orbital inflammatory pseudotumour (OIP) with intracranial extension, a refractory and high-relapse disease. Methods: Patients who had been diagnosed with refractory OIP with intracranial extension and who were refractory to systemic corticosteroids were retrospectively recruited at the Neuro-Ophthalmology Department at the Chinese People’s Liberation Army General Hospital between December 2018 and September 2022. After methylprednisolone pulse therapy, we added 2 mg of tacrolimus per day, 1500 mg of mycophenolate mofetil per day, or 200 mg of rituximab at days 1 and 15, and then monitored those with CD19+ B cells of under 1% as adjuvant therapy. Results: Eleven patients (six males and five females) were included, with a mean age of 45.5±11.8 years (age range: 21–64 years). The average follow-up period was 3.8 years (range: 2–5). Eight patients (72.7%) had different levels of decreased vision at onset of the illness and four patients (36.4%) had severely impaired vision (three with no light perception, one with some light perception). Four patients (36.4%) showed clinical course worsening or lack of remission when treated with corticosteroids. Seven patients (63.6%) had a typical relapsing course, and the annual recurrence rate was higher than 7.36±3.73 times. Of these seven, four (57.1%, 4/7) were able to undergo successful management with immunosuppressants. Three (42.9%, 3/7) failed with immunosuppressants but succeeded in controlling relapse with RTX. Conclusion: OIP with intracranial extension is uncommon. More than half of patients with OIP with intracranial extension may be satisfactorily treated with corticosteroids combined with immunosuppressants. However, for patients who still experience recurrence or slow reduction of lesions after applying this combined therapy, RTX may be a better option.

    Keywords: orbital inflammatory pseudotumors, inflammation with intracranial extension, Treatment, rituximab, Immunosuppressants

    Received: 25 Oct 2024; Accepted: 07 Jan 2025.

    Copyright: © 2025 Li, Sun, Chen, Xu, Wang, Xu, Zhou and Shihui. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Huanfen Zhou, Department of Ophthalmology, The Third Medical Center of Chinese PLA General Hospital, Yuzhong, 400042, Chongqing, China
    Wei Shihui, Department of Ophthalmology, The Third Medical Center of Chinese PLA General Hospital, Yuzhong, 400042, Chongqing, China

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