Albahra Said, Yates Sean G, Joseph David, De Simone Nicole, Burner James D, Sarode Ravi
Department of Pathology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390-9072, USA.
Department of Pathology, Division of Transfusion Medicine, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX 77555, USA.
Transfus Apher Sci. 2019 Jun;58(3):310-312. doi: 10.1016/j.transci.2019.03.015. Epub 2019 Mar 19.
Stiff person syndrome (SPS) is commonly associated with antibodies directed against 65-kDa glutamic acid decarboxylase (GAD65). Therapeutic Plasma Exchange (TPE) has been used as an adjunct therapy in patients who do not respond well to conventional treatment, which includes immunosuppression therapies, anti-anxiety medications, muscle relaxants, anticonvulsants, and pain relievers.
We retrospectively analyzed the clinical data and outcomes of ten patients with the clinical diagnosis of anti-GAD65 positive SPS in which TPE was employed to improve symptoms refractory to conventional treatment during an eight-year period.
TPE was initiated as complementary therapy in patients with worsening of symptoms characteristic of SPS. Six patients underwent chronic treatment with TPE following an initial course, of which the frequency of TPE was guided by the clinical response. Two patients only had transient improvements with further disease progression. Four patients developed a relapse of symptoms when the interval between procedures was increased. One of the four patients dependent on TPE had worsening of symptoms following complete cessation of TPE due to lack of insurance coverage. Four patients underwent only an acute hospitalized course of treatment with TPE; one demonstrated complete resolution of symptoms; one had a partial response; and two experienced no improvement.
Our study supports previous reports that TPE may be beneficial for the management of patients with anti-GAD65 positive SPS, both for acute exacerbations and long-term maintenance, either as an adjunct therapy, or in lieu of treatment with disease modifying agents.
僵人综合征（SPS）通常与针对65kDa谷氨酸脱羧酶（GAD65）的抗体相关。治疗性血浆置换（TPE）已被用作对传统治疗反应不佳患者的辅助治疗，传统治疗包括免疫抑制疗法、抗焦虑药物、肌肉松弛剂、抗惊厥药和止痛药。
我们回顾性分析了10例临床诊断为抗GAD65阳性SPS患者的临床资料和结局，这些患者在8年期间采用TPE来改善对传统治疗难治的症状。
TPE作为SPS特征性症状恶化患者的补充治疗开始使用。6例患者在初始疗程后接受了TPE的长期治疗，其中TPE的频率由临床反应指导。2例患者仅出现短暂改善，随后疾病进展。4例患者在治疗间隔增加时症状复发。4例依赖TPE的患者中有1例因缺乏保险覆盖在完全停止TPE后症状恶化。4例患者仅接受了TPE的急性住院治疗疗程；1例症状完全缓解；1例部分缓解；2例无改善。
我们的研究支持先前的报道，即TPE可能有益于抗GAD65阳性SPS患者的管理，无论是作为辅助治疗还是替代疾病修饰药物治疗，对急性加重期和长期维持治疗均有益。
