Xue Chen, MingFang Sun, Ling Wang, YanLong Yang, HuanZi Dai
{"title":"Response to telitacicept in optic neuritis associated with Sjogren's syndrome: a case report and literature review.","authors":"Xue Chen, MingFang Sun, Ling Wang, YanLong Yang, HuanZi Dai","doi":"10.1007/s00415-025-13099-9","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Optic neuritis (ON) frequently presents as the initial symptom of multiple sclerosis and neuromyelitis optica. Additionally, ON is one of the manifestations of central nervous system damage in Sjögren's syndrome (SS). However, there is little research on SS-associated ON. Currently, there are no standard treatment methods for SS-associated ON, and the recurrence rate remains high.</p><p><strong>Methods: </strong>A 54-year-old female patient primarily exhibited symptoms of dry mouth and dry eyes, along with rapidly deteriorating vision and pain in the left eye. Laboratory tests revealed strong positivity for anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies, and a labial salivary gland biopsy showed focal lymphocytic sialadenitis with a focus score of 13 per 4 mm<sup>2</sup>. Visual evoked potentials indicated a significant absence of waveform in the left eye. The patient was diagnosed with SS-associated ON; however, treatment with glucocorticoid (GC) and cyclophosphamide was unsatisfactory. The patient declined plasma exchange and Rituximab therapy; consequently, she was treated with GC and telitacicept.</p><p><strong>Results: </strong>During a 9-month follow-up period, at the minimum GC dosage, the patient's symptoms of dryness and visual acuity in the left eye improved significantly, with no reported recurrence or adverse drug reactions. This indicates both good clinical efficacy and safety.</p><p><strong>Conclusion: </strong>SS-associated ON is uncommon and easily misdiagnosed. Conventional therapies for ON cannot control recurrence. Telitacicept might represent a promising choice to treat SS-associated ON.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 7","pages":"462"},"PeriodicalIF":4.8000,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00415-025-13099-9","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Optic neuritis (ON) frequently presents as the initial symptom of multiple sclerosis and neuromyelitis optica. Additionally, ON is one of the manifestations of central nervous system damage in Sjögren's syndrome (SS). However, there is little research on SS-associated ON. Currently, there are no standard treatment methods for SS-associated ON, and the recurrence rate remains high.
Methods: A 54-year-old female patient primarily exhibited symptoms of dry mouth and dry eyes, along with rapidly deteriorating vision and pain in the left eye. Laboratory tests revealed strong positivity for anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies, and a labial salivary gland biopsy showed focal lymphocytic sialadenitis with a focus score of 13 per 4 mm2. Visual evoked potentials indicated a significant absence of waveform in the left eye. The patient was diagnosed with SS-associated ON; however, treatment with glucocorticoid (GC) and cyclophosphamide was unsatisfactory. The patient declined plasma exchange and Rituximab therapy; consequently, she was treated with GC and telitacicept.
Results: During a 9-month follow-up period, at the minimum GC dosage, the patient's symptoms of dryness and visual acuity in the left eye improved significantly, with no reported recurrence or adverse drug reactions. This indicates both good clinical efficacy and safety.
Conclusion: SS-associated ON is uncommon and easily misdiagnosed. Conventional therapies for ON cannot control recurrence. Telitacicept might represent a promising choice to treat SS-associated ON.
期刊介绍:
The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field.
In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials.
Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.