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Therapeutic plasma exchange in the management of stiff person syndrome spectrum disorders: a case series and review of the literature.
Mercure-Corriveau, Nicolas; Roy, Shuvro; Hu, Chen; Crowe, Elizabeth P; Zhu, Xianming; Obando, Danielle; Patel, Eshan U; Tobian, Aaron A R; Wang, Yujie; Bloch, Evan M; Newsome, Scott D.
Afiliación
  • Mercure-Corriveau N; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Roy S; Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University, School of Medicine, Baltimore, MD, USA.
  • Hu C; Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University, School of Medicine, Baltimore, MD, USA.
  • Crowe EP; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Zhu X; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Obando D; Department of Neurology, Johns Hopkins University, School of Medicine, Baltimore, MD, USA.
  • Patel EU; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Tobian AAR; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Wang Y; Department of Neurology, Johns Hopkins University, School of Medicine, Baltimore, MD, USA.
  • Bloch EM; Division of Transfusion Medicine, Department of Pathology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Newsome SD; Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University, School of Medicine, 600 North Wolfe Street, Pathology 627, Baltimore, MD 21287, USA.
Ther Adv Neurol Disord ; 16: 17562864231180736, 2023.
Article en En | MEDLINE | ID: mdl-37529719
ABSTRACT

Background:

Stiff person syndrome spectrum disorders (SPSD) are a rare group of disabling neuroimmunological disorders. SPSD often requires immune therapies, especially in the setting of inadequate response to symptomatic treatments. The safety and efficacy of therapeutic plasma exchange (TPE) in SPSD remains uncertain.

Objectives:

To describe the safety, tolerability, and efficacy of TPE in patients with SPSD.

Design:

A retrospective observational study.

Methods:

A retrospective review of SPSD patients seen at Johns Hopkins Hospital (JHH) from 1997 to 2021 was performed. Patient demographics/history, examination/diagnostic findings, treatment response, and TPE-related complications were recorded. Assessment for any associations between clinical characteristics, including age, sex, clinical phenotype, and time on immunotherapy, and response to TPE 3 months after treatment was performed. A subgroup of 18 patients treated with TPE at JHH and 6 patients treated with TPE at outside institutions were evaluated for any change in usage of symptomatic medications 3 months after the TPE treatment. Literature review of SPSD and TPE was also conducted.

Results:

Thirty-nine SPSD patients were treated with TPE (21 at JHH and 18 at outside institutions); median age 48 years, 77% female, median modified Rankin Scale 3; mean initial anti-GAD65 antibody titer was 23,508 U/mL. Twenty-four patients (62%) had classic SPS, 10 (26%) had SPS-plus, 2 (5%) had progressive encephalomyelitis with rigidity and myoclonus, and 3 (8%) had pure cerebellar ataxia. All patients were on symptomatic treatments, 30 (77%) previously received IVIg, and 3 (8%) previously received rituximab. Four patients (10%) had a TPE-related adverse event. One developed asymptomatic hypotension, another had both line thrombosis and infection, and two had non-life-threatening bleeding events. Twenty-three (59%) patients reported improvement in symptoms after TPE. Of the subgroup of 24 patients evaluated for any change in usage of symptomatic medications 3 months after the TPE treatment, 14 (58%) required fewer GABAergic symptomatic medications. Literature review identified 57 additional patients with SPSD; 43 (75%) reported temporary improvement after TPE.

Conclusion:

The majority of patients treated with TPE had improvement. Moreover, most patients evaluated for any change in usage of symptomatic medications after the TPE treatment no longer required as much symptomatic medications months after TPE. TPE appears safe and well-tolerated in SPSD. Further studies are needed to assess the long-term efficacy of TPE in SPSD and identify which patients may benefit the most from TPE.
Palabras clave

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Tipo de estudio: Observational_studies / Prognostic_studies Idioma: En Revista: Ther Adv Neurol Disord Año: 2023 Tipo del documento: Article País de afiliación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Tipo de estudio: Observational_studies / Prognostic_studies Idioma: En Revista: Ther Adv Neurol Disord Año: 2023 Tipo del documento: Article País de afiliación: Estados Unidos