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

ObjectiveThe aim of this study was to compare the efficacy of low-dose rituximab (RTX) and immunosuppressants in treating orbital inflammatory pseudotumor (OIP) with intracranial extension, a refractory and high-relapse disease.MethodsPatients who had been diagnosed with refractory OIP with intracra...

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Main Authors: Yuyu Li, Mingming Sun, Xintong Xu, Biyue Chen, Xiyun Chen, Yuhang Wang, Quangang Xu, Huanfen Zhou, Shihui Wei
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-01-01
Series:Frontiers in Immunology
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Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2025.1516909/full
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author Yuyu Li
Mingming Sun
Xintong Xu
Biyue Chen
Xiyun Chen
Yuhang Wang
Quangang Xu
Huanfen Zhou
Shihui Wei
author_facet Yuyu Li
Mingming Sun
Xintong Xu
Biyue Chen
Xiyun Chen
Yuhang Wang
Quangang Xu
Huanfen Zhou
Shihui Wei
author_sort Yuyu Li
collection DOAJ
description ObjectiveThe aim of this study was to compare the efficacy of low-dose rituximab (RTX) and immunosuppressants in treating orbital inflammatory pseudotumor (OIP) with intracranial extension, a refractory and high-relapse disease.MethodsPatients 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.ResultsEleven 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.ConclusionOIP 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.
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spelling doaj-art-22fb78c9490e4041b14a6531305ebb642025-01-24T07:13:27ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-01-011610.3389/fimmu.2025.15169091516909Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extensionYuyu LiMingming SunXintong XuBiyue ChenXiyun ChenYuhang WangQuangang XuHuanfen ZhouShihui WeiObjectiveThe aim of this study was to compare the efficacy of low-dose rituximab (RTX) and immunosuppressants in treating orbital inflammatory pseudotumor (OIP) with intracranial extension, a refractory and high-relapse disease.MethodsPatients 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.ResultsEleven 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.ConclusionOIP 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.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1516909/fullorbital inflammatory pseudotumorsinflammation with intracranial extensiontreatmentrituximabimmunosuppressants
spellingShingle Yuyu Li
Mingming Sun
Xintong Xu
Biyue Chen
Xiyun Chen
Yuhang Wang
Quangang Xu
Huanfen Zhou
Shihui Wei
Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
Frontiers in Immunology
orbital inflammatory pseudotumors
inflammation with intracranial extension
treatment
rituximab
immunosuppressants
title Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
title_full Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
title_fullStr Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
title_full_unstemmed Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
title_short Efficacy of low-dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
title_sort efficacy of low dose rituximab versus immunosuppressants in refractory orbital inflammatory pseudotumors with intracranial extension
topic orbital inflammatory pseudotumors
inflammation with intracranial extension
treatment
rituximab
immunosuppressants
url https://www.frontiersin.org/articles/10.3389/fimmu.2025.1516909/full
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