Pignolo Antonia, Vinciguerra Claudia, Monastero Roberto, Rini Nicasio, Torrente Angelo, Balistreri Carmela Rita, Brighina Filippo, Di Stefano Vincenzo
Department of Biomedicine, Neuroscience, and Advanced Diagnostics (Bi.N.D.), University of Palermo, 90129, Palermo, Italy.
Neurology Unit, Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University Hospital San Giovanni Di Dio E Ruggi D'Aragona, 84131, Salerno, Italy.
J Neurol. 2025 May 24;272(6):417. doi: 10.1007/s00415-025-13157-2.
Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder characterized by muscle rigidity and painful spasms, predominantly affecting young women. It is often associated with high titers of anti-glutamic acid decarboxylase (GAD) 65 antibodies. Current treatments for SPS include symptomatic therapies and immunomodulatory approaches, but there is a need for more effective therapies because many patients show incomplete responses and disease progression.
The systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, with a literature search of PubMed, Web of Knowledge, Google Scholar, and Science Direct. Studies evaluating efficacy, safety, dosage, and impact on concomitant treatments of Rituximab (RTX) in SPS were selected. Data on anti-GAD titers were also analyzed.
Fourteen studies published between July 2005 and October 2022 were selected. The studies included 30 SPS patients treated with RTX. Data were heterogeneous regarding dosage, administration schedule, and patient assessment. RTX was generally well-tolerated, with rare side effects, including infusion reactions or infections. Significant clinical improvement occurred in most patients, with a small proportion achieving complete remission. Anti-GAD antibody titers decreased in some studies, with no consistent correlation with clinical outcomes.
Evidence supporting the efficacy of RTX in SPS is limited by the small sample sizes of the included studies and the variability in treatment protocols. However, RTX has shown efficacy for clinical improvement. Correlation with anti-GAD titers remains still unclear. Further randomized controlled trials are needed to confirm RTX as an established treatment for SPS.
僵人综合征（SPS）是一种罕见的自身免疫性神经疾病，其特征为肌肉僵硬和疼痛性痉挛，主要影响年轻女性。它常与高滴度的抗谷氨酸脱羧酶（GAD）65抗体相关。目前SPS的治疗方法包括对症治疗和免疫调节方法，但由于许多患者反应不完全且疾病仍有进展，因此需要更有效的治疗方法。
本系统评价遵循系统评价和Meta分析的首选报告项目（PRISMA）指南，通过在PubMed、Web of Knowledge、谷歌学术和科学Direct上进行文献检索。选择评估利妥昔单抗（RTX）治疗SPS的疗效、安全性、剂量以及对伴随治疗影响的研究。还分析了抗GAD滴度的数据。
选取了2005年7月至2022年10月间发表的14项研究。这些研究纳入了30例接受RTX治疗的SPS患者。在剂量、给药方案和患者评估方面，数据存在异质性。RTX总体耐受性良好，副作用罕见，包括输液反应或感染。大多数患者出现了显著的临床改善，一小部分患者实现了完全缓解。在一些研究中抗GAD抗体滴度有所下降，但与临床结果无一致的相关性。
纳入研究的样本量较小以及治疗方案的变异性限制了支持RTX治疗SPS疗效的证据。然而，RTX已显示出临床改善的疗效。与抗GAD滴度的相关性仍不明确。需要进一步的随机对照试验来证实RTX作为SPS既定治疗方法的有效性。
