Excellent response to therapeutic plasma exchange in myasthenia gravis patients irrespective of antibody status

Amena Usmani, Laura Kwan, Dina Wahib-Khalil, Jaya R Trivedi, Sharon P Nations, Ravindra Sarode

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Abstract

Introduction: The primary objective of this study was to assess response to plasma exchange (PLEX) in myasthenia gravis (MG) patients with and without autoantibodies (Ab) to acetylcholine receptor (AChR) or muscle-specific kinase (MuSK). Analysis was also done to determine if correlation existed between sex, early or late onset MG, thymoma, or thymectomy and response to PLEX. Materials and Methods: Data was analyzed on 58 consecutive MG patients treated with PLEX. Responses were categorized as complete response, clinical improvement requiring maintenance PLEX, or no/minimal response to PLEX. Results: Eighty-eight percent (51/58) of patients were Ab-positive; 44 had AChR and 7 had MuSK Ab. Complete response was seen in 26 patients (24 Ab+), 24 remain on maintenance PLEX (19 Ab+), and 2 had no/minimal response (both AChR Ab+). Ab status (P = 0.43), AChR Ab (P = 0.10), MuSK Ab (P = 0.45), early onset MG (P = 0.63), thymoma (P = 0.46), and thymectomy (P = 0.16) were not significantly associated with outcome. Patient sex did show significant association with outcome (P = 0.01), with men more likely to have complete response and women more likely to require maintenance. Late onset MG is significantly associated with higher likelihood of complete response (P = 0.03). Antibody titers declined after PLEX in 83% of patients with complete response, in whom pre- and post-PLEX titers were available (n = 6). Conclusions: In conclusion, our study showed 96% response rate to PLEX in MG; however, only patient gender and late onset MG were significantly associated with treatment response.

Original languageEnglish (US)
JournalJournal of Clinical Apheresis
DOIs
StatePublished - Jan 1 2019

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Plasma Exchange
Myasthenia Gravis
Autoantibodies
Antibodies
Cholinergic Receptors
Thymectomy
Phosphotransferases
Thymoma
Therapeutics
Maintenance
Muscles

Keywords

  • acetylcholine receptor antibody (AChR)
  • muscle specific kinase (MuSK) antibody
  • myasthenia gravis (MG)
  • plasma exchange

ASJC Scopus subject areas

  • Hematology

Cite this

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title = "Excellent response to therapeutic plasma exchange in myasthenia gravis patients irrespective of antibody status",
abstract = "Introduction: The primary objective of this study was to assess response to plasma exchange (PLEX) in myasthenia gravis (MG) patients with and without autoantibodies (Ab) to acetylcholine receptor (AChR) or muscle-specific kinase (MuSK). Analysis was also done to determine if correlation existed between sex, early or late onset MG, thymoma, or thymectomy and response to PLEX. Materials and Methods: Data was analyzed on 58 consecutive MG patients treated with PLEX. Responses were categorized as complete response, clinical improvement requiring maintenance PLEX, or no/minimal response to PLEX. Results: Eighty-eight percent (51/58) of patients were Ab-positive; 44 had AChR and 7 had MuSK Ab. Complete response was seen in 26 patients (24 Ab+), 24 remain on maintenance PLEX (19 Ab+), and 2 had no/minimal response (both AChR Ab+). Ab status (P = 0.43), AChR Ab (P = 0.10), MuSK Ab (P = 0.45), early onset MG (P = 0.63), thymoma (P = 0.46), and thymectomy (P = 0.16) were not significantly associated with outcome. Patient sex did show significant association with outcome (P = 0.01), with men more likely to have complete response and women more likely to require maintenance. Late onset MG is significantly associated with higher likelihood of complete response (P = 0.03). Antibody titers declined after PLEX in 83{\%} of patients with complete response, in whom pre- and post-PLEX titers were available (n = 6). Conclusions: In conclusion, our study showed 96{\%} response rate to PLEX in MG; however, only patient gender and late onset MG were significantly associated with treatment response.",
keywords = "acetylcholine receptor antibody (AChR), muscle specific kinase (MuSK) antibody, myasthenia gravis (MG), plasma exchange",
author = "Amena Usmani and Laura Kwan and Dina Wahib-Khalil and Trivedi, {Jaya R} and Nations, {Sharon P} and Ravindra Sarode",
year = "2019",
month = "1",
day = "1",
doi = "10.1002/jca.21694",
language = "English (US)",
journal = "Journal of Clinical Apheresis",
issn = "0733-2459",
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TY - JOUR

T1 - Excellent response to therapeutic plasma exchange in myasthenia gravis patients irrespective of antibody status

AU - Usmani, Amena

AU - Kwan, Laura

AU - Wahib-Khalil, Dina

AU - Trivedi, Jaya R

AU - Nations, Sharon P

AU - Sarode, Ravindra

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Introduction: The primary objective of this study was to assess response to plasma exchange (PLEX) in myasthenia gravis (MG) patients with and without autoantibodies (Ab) to acetylcholine receptor (AChR) or muscle-specific kinase (MuSK). Analysis was also done to determine if correlation existed between sex, early or late onset MG, thymoma, or thymectomy and response to PLEX. Materials and Methods: Data was analyzed on 58 consecutive MG patients treated with PLEX. Responses were categorized as complete response, clinical improvement requiring maintenance PLEX, or no/minimal response to PLEX. Results: Eighty-eight percent (51/58) of patients were Ab-positive; 44 had AChR and 7 had MuSK Ab. Complete response was seen in 26 patients (24 Ab+), 24 remain on maintenance PLEX (19 Ab+), and 2 had no/minimal response (both AChR Ab+). Ab status (P = 0.43), AChR Ab (P = 0.10), MuSK Ab (P = 0.45), early onset MG (P = 0.63), thymoma (P = 0.46), and thymectomy (P = 0.16) were not significantly associated with outcome. Patient sex did show significant association with outcome (P = 0.01), with men more likely to have complete response and women more likely to require maintenance. Late onset MG is significantly associated with higher likelihood of complete response (P = 0.03). Antibody titers declined after PLEX in 83% of patients with complete response, in whom pre- and post-PLEX titers were available (n = 6). Conclusions: In conclusion, our study showed 96% response rate to PLEX in MG; however, only patient gender and late onset MG were significantly associated with treatment response.

AB - Introduction: The primary objective of this study was to assess response to plasma exchange (PLEX) in myasthenia gravis (MG) patients with and without autoantibodies (Ab) to acetylcholine receptor (AChR) or muscle-specific kinase (MuSK). Analysis was also done to determine if correlation existed between sex, early or late onset MG, thymoma, or thymectomy and response to PLEX. Materials and Methods: Data was analyzed on 58 consecutive MG patients treated with PLEX. Responses were categorized as complete response, clinical improvement requiring maintenance PLEX, or no/minimal response to PLEX. Results: Eighty-eight percent (51/58) of patients were Ab-positive; 44 had AChR and 7 had MuSK Ab. Complete response was seen in 26 patients (24 Ab+), 24 remain on maintenance PLEX (19 Ab+), and 2 had no/minimal response (both AChR Ab+). Ab status (P = 0.43), AChR Ab (P = 0.10), MuSK Ab (P = 0.45), early onset MG (P = 0.63), thymoma (P = 0.46), and thymectomy (P = 0.16) were not significantly associated with outcome. Patient sex did show significant association with outcome (P = 0.01), with men more likely to have complete response and women more likely to require maintenance. Late onset MG is significantly associated with higher likelihood of complete response (P = 0.03). Antibody titers declined after PLEX in 83% of patients with complete response, in whom pre- and post-PLEX titers were available (n = 6). Conclusions: In conclusion, our study showed 96% response rate to PLEX in MG; however, only patient gender and late onset MG were significantly associated with treatment response.

KW - acetylcholine receptor antibody (AChR)

KW - muscle specific kinase (MuSK) antibody

KW - myasthenia gravis (MG)

KW - plasma exchange

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U2 - 10.1002/jca.21694

DO - 10.1002/jca.21694

M3 - Article

C2 - 30779438

AN - SCOPUS:85061930711

JO - Journal of Clinical Apheresis

JF - Journal of Clinical Apheresis

SN - 0733-2459

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