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Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet’s syndrome: case series and literature review
OBJECTIVES: This study aimed to investigate the efficacy and safety of tocilizumab (TCZ) in severe and refractory parenchymal neuro-Behçet’s syndrome (p-NBS). METHODS: We retrospectively analyzed five patients with p-NBS treated with TCZ in our center between 2013 and 2020, and six cases from litera...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7675870/ https://www.ncbi.nlm.nih.gov/pubmed/33240406 http://dx.doi.org/10.1177/1759720X20971908 |
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author | Liu, Jinjing Yan, Dong Wang, Zhimian Yang, Yunjiao Zhang, Shangzhu Wu, Di Peng, Lingyi Liu, Zhichun Zheng, Wenjie |
author_facet | Liu, Jinjing Yan, Dong Wang, Zhimian Yang, Yunjiao Zhang, Shangzhu Wu, Di Peng, Lingyi Liu, Zhichun Zheng, Wenjie |
author_sort | Liu, Jinjing |
collection | PubMed |
description | OBJECTIVES: This study aimed to investigate the efficacy and safety of tocilizumab (TCZ) in severe and refractory parenchymal neuro-Behçet’s syndrome (p-NBS). METHODS: We retrospectively analyzed five patients with p-NBS treated with TCZ in our center between 2013 and 2020, and six cases from literature research with the index terms “neuro-Behçet’s syndrome” and “tocilizumab” on PubMed NCBI. RESULTS: A total of 11 patients with p-NBS were enrolled (5 males, 6 females), with a mean age of 34.5 ± 8.0 years at the onset. All the patients had parenchymal neurological lesions, six patients (54.5%) suffered from multiple lesions, and nine patients (81.8%) were disabled. Before TCZ administration, all the patients had failed conventional therapy, eight patients (72.7%) received two or more immunosuppressants, and five patients showed insufficient response or intolerance to other biologics. TCZ was administrated at 8 mg/kg every 4 weeks, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 13 (interquartile range, 3.5–23.5) months, all the patients achieved both clinical and radiological improvements, and the Behçet’s Disease Current Activity Form score improved significantly (3 versus 0, median, p = 0.004), the Rankin score also decreased (4 versus 2, median, p = 0.005). Levels of interleukin-6 in the cerebrospinal fluid decreased significantly in five patients (533.4 ± 389.7 pg/ml versus 34.5 ± 27.1 pg/ml, p = 0.048), after a median of two (interquartile range, 1–4) times of TCZ infusions. Furthermore, the GC dosage (per os) reduced from 69.2 ± 16.9 mg/d to 16.4 ± 16.2 mg/d (p = 0.000), and immunosuppressants were tapered in number and dosage in seven (63.6%) and four (36.3%) patients, respectively. No serious adverse events or deaths were observed during follow-up. CONCLUSIONS: TCZ is well tolerated and effective in severe and refractory p-NBS, with a favorable GC- and immunosuppressant-sparing effect. Cerebrospinal fluid interleukin-6 might be used to monitor the effects of TCZ in p-NBS. |
format | Online Article Text |
id | pubmed-7675870 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-76758702020-11-24 Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet’s syndrome: case series and literature review Liu, Jinjing Yan, Dong Wang, Zhimian Yang, Yunjiao Zhang, Shangzhu Wu, Di Peng, Lingyi Liu, Zhichun Zheng, Wenjie Ther Adv Musculoskelet Dis Case Series OBJECTIVES: This study aimed to investigate the efficacy and safety of tocilizumab (TCZ) in severe and refractory parenchymal neuro-Behçet’s syndrome (p-NBS). METHODS: We retrospectively analyzed five patients with p-NBS treated with TCZ in our center between 2013 and 2020, and six cases from literature research with the index terms “neuro-Behçet’s syndrome” and “tocilizumab” on PubMed NCBI. RESULTS: A total of 11 patients with p-NBS were enrolled (5 males, 6 females), with a mean age of 34.5 ± 8.0 years at the onset. All the patients had parenchymal neurological lesions, six patients (54.5%) suffered from multiple lesions, and nine patients (81.8%) were disabled. Before TCZ administration, all the patients had failed conventional therapy, eight patients (72.7%) received two or more immunosuppressants, and five patients showed insufficient response or intolerance to other biologics. TCZ was administrated at 8 mg/kg every 4 weeks, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 13 (interquartile range, 3.5–23.5) months, all the patients achieved both clinical and radiological improvements, and the Behçet’s Disease Current Activity Form score improved significantly (3 versus 0, median, p = 0.004), the Rankin score also decreased (4 versus 2, median, p = 0.005). Levels of interleukin-6 in the cerebrospinal fluid decreased significantly in five patients (533.4 ± 389.7 pg/ml versus 34.5 ± 27.1 pg/ml, p = 0.048), after a median of two (interquartile range, 1–4) times of TCZ infusions. Furthermore, the GC dosage (per os) reduced from 69.2 ± 16.9 mg/d to 16.4 ± 16.2 mg/d (p = 0.000), and immunosuppressants were tapered in number and dosage in seven (63.6%) and four (36.3%) patients, respectively. No serious adverse events or deaths were observed during follow-up. CONCLUSIONS: TCZ is well tolerated and effective in severe and refractory p-NBS, with a favorable GC- and immunosuppressant-sparing effect. Cerebrospinal fluid interleukin-6 might be used to monitor the effects of TCZ in p-NBS. SAGE Publications 2020-11-16 /pmc/articles/PMC7675870/ /pubmed/33240406 http://dx.doi.org/10.1177/1759720X20971908 Text en © The Author(s), 2020 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Series Liu, Jinjing Yan, Dong Wang, Zhimian Yang, Yunjiao Zhang, Shangzhu Wu, Di Peng, Lingyi Liu, Zhichun Zheng, Wenjie Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet’s syndrome: case series and literature review |
title | Tocilizumab in the treatment of severe and refractory parenchymal
neuro-Behçet’s syndrome: case series and literature review |
title_full | Tocilizumab in the treatment of severe and refractory parenchymal
neuro-Behçet’s syndrome: case series and literature review |
title_fullStr | Tocilizumab in the treatment of severe and refractory parenchymal
neuro-Behçet’s syndrome: case series and literature review |
title_full_unstemmed | Tocilizumab in the treatment of severe and refractory parenchymal
neuro-Behçet’s syndrome: case series and literature review |
title_short | Tocilizumab in the treatment of severe and refractory parenchymal
neuro-Behçet’s syndrome: case series and literature review |
title_sort | tocilizumab in the treatment of severe and refractory parenchymal
neuro-behçet’s syndrome: case series and literature review |
topic | Case Series |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7675870/ https://www.ncbi.nlm.nih.gov/pubmed/33240406 http://dx.doi.org/10.1177/1759720X20971908 |
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