Role of plasma exchange in stiff person syndrome

Said Albahra, Sean Yates, David Joseph, Nicole De Simone, James D. Burner, Ravi Sarode

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objective: 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. Methods: 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. Results: 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. Conclusion: 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.

Original languageEnglish (US)
JournalTransfusion and Apheresis Science
DOIs
StatePublished - Jan 1 2019

Fingerprint

Stiff-Person Syndrome
Plasma Exchange
Therapeutics

Keywords

  • Stiff person syndrome
  • Therapeutic plasma exchange

ASJC Scopus subject areas

  • Hematology

Cite this

Role of plasma exchange in stiff person syndrome. / Albahra, Said; Yates, Sean; Joseph, David; De Simone, Nicole; Burner, James D.; Sarode, Ravi.

In: Transfusion and Apheresis Science, 01.01.2019.

Research output: Contribution to journalArticle

Albahra, Said ; Yates, Sean ; Joseph, David ; De Simone, Nicole ; Burner, James D. ; Sarode, Ravi. / Role of plasma exchange in stiff person syndrome. In: Transfusion and Apheresis Science. 2019.
@article{b0b36cbf7ac749fa847f428574c9f4a4,
title = "Role of plasma exchange in stiff person syndrome",
abstract = "Objective: 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. Methods: 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. Results: 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. Conclusion: 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.",
keywords = "Stiff person syndrome, Therapeutic plasma exchange",
author = "Said Albahra and Sean Yates and David Joseph and {De Simone}, Nicole and Burner, {James D.} and Ravi Sarode",
year = "2019",
month = "1",
day = "1",
doi = "10.1016/j.transci.2019.03.015",
language = "English (US)",
journal = "Transfusion and Apheresis Science",
issn = "1473-0502",
publisher = "Elsevier Limited",

}

TY - JOUR

T1 - Role of plasma exchange in stiff person syndrome

AU - Albahra, Said

AU - Yates, Sean

AU - Joseph, David

AU - De Simone, Nicole

AU - Burner, James D.

AU - Sarode, Ravi

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Objective: 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. Methods: 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. Results: 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. Conclusion: 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.

AB - Objective: 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. Methods: 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. Results: 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. Conclusion: 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.

KW - Stiff person syndrome

KW - Therapeutic plasma exchange

UR - http://www.scopus.com/inward/record.url?scp=85063639588&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85063639588&partnerID=8YFLogxK

U2 - 10.1016/j.transci.2019.03.015

DO - 10.1016/j.transci.2019.03.015

M3 - Article

C2 - 30952585

AN - SCOPUS:85063639588

JO - Transfusion and Apheresis Science

JF - Transfusion and Apheresis Science

SN - 1473-0502

ER -