AChR-blocking antibodies and complement system dynamics: evaluating their interplay and clinical implications in myasthenia gravis.

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Title: AChR-blocking antibodies and complement system dynamics: evaluating their interplay and clinical implications in myasthenia gravis.
Authors: Aguirre, Florencia (AUTHOR), Justo, Mariano E. (AUTHOR), Cialdella, Lucía (AUTHOR), Paz, Mariela L. (AUTHOR)
Source: Neurological Sciences. Apr2025, Vol. 46 Issue 4, p1827-1832. 6p.
Subjects: Myasthenia gravis, Complement activation, Cholinergic receptors, Myoneural junction, Inverse relationships (Mathematics)
Abstract: Background: Myasthenia gravis (MG) is an autoimmune disorder characterised by autoantibodies (abs) targeting proteins at the neuromuscular junction, primarily the acetylcholine receptor (AChR). While the role of AChR-binding abs is well-established, the pathogenicity and clinical relevance of AChR-blocking antibodies in MG, and their association with complement system, remain less understood. Aims: This study aims to provide comprehensive insights into the prevalence and interplay of AChR-blocking antibodies and the complement system in an Argentinian MG cohort, investigating their relationships with disease activity. Methods: We studied 75 MG patients with detectable AChR-binding abs, assessing the presence of AChR-blocking abs and complement components C3, C4, and C5a. We also examined clinical severity using the Activities of Daily Living and MG Composite scores. Correlation analyses were made to elucidate associations. Results: AChR-blocking abs were detected in 49.3% of the patients. An inverse correlation was found between AChR-blocking abs titres and disease severity, with a higher titre associated with milder symptoms. Complement analysis revealed higher C4 levels in the AChR-blocking abs positive group, indicating reduced complement activation. Conclusion: Our study provides valuable insights into the prevalence of AChR-blocking antibodies. Higher AChR-blocking abs titres were associated with less severe MG and reduced complement system activation, indicating a potential protective mechanism for those abs. These findings suggest that AChR-blocking abs could serve as a potential biomarker for a milder disease course and highlight the need for further research to understand their role in MG pathology, which will improve strategies for clinical management and diagnosis. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: AChR-blocking antibodies and complement system dynamics: evaluating their interplay and clinical implications in myasthenia gravis.
– Name: Author
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  Data: <searchLink fieldCode="AR" term="%22Aguirre%2C+Florencia%22">Aguirre, Florencia</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Justo%2C+Mariano+E%2E%22">Justo, Mariano E.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Cialdella%2C+Lucía%22">Cialdella, Lucía</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Paz%2C+Mariela+L%2E%22">Paz, Mariela L.</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. Apr2025, Vol. 46 Issue 4, p1827-1832. 6p.
– Name: Subject
  Label: Subjects
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22Myasthenia+gravis%22">Myasthenia gravis</searchLink><br /><searchLink fieldCode="DE" term="%22Complement+activation%22">Complement activation</searchLink><br /><searchLink fieldCode="DE" term="%22Cholinergic+receptors%22">Cholinergic receptors</searchLink><br /><searchLink fieldCode="DE" term="%22Myoneural+junction%22">Myoneural junction</searchLink><br /><searchLink fieldCode="DE" term="%22Inverse+relationships+%28Mathematics%29%22">Inverse relationships (Mathematics)</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: Myasthenia gravis (MG) is an autoimmune disorder characterised by autoantibodies (abs) targeting proteins at the neuromuscular junction, primarily the acetylcholine receptor (AChR). While the role of AChR-binding abs is well-established, the pathogenicity and clinical relevance of AChR-blocking antibodies in MG, and their association with complement system, remain less understood. Aims: This study aims to provide comprehensive insights into the prevalence and interplay of AChR-blocking antibodies and the complement system in an Argentinian MG cohort, investigating their relationships with disease activity. Methods: We studied 75 MG patients with detectable AChR-binding abs, assessing the presence of AChR-blocking abs and complement components C3, C4, and C5a. We also examined clinical severity using the Activities of Daily Living and MG Composite scores. Correlation analyses were made to elucidate associations. Results: AChR-blocking abs were detected in 49.3% of the patients. An inverse correlation was found between AChR-blocking abs titres and disease severity, with a higher titre associated with milder symptoms. Complement analysis revealed higher C4 levels in the AChR-blocking abs positive group, indicating reduced complement activation. Conclusion: Our study provides valuable insights into the prevalence of AChR-blocking antibodies. Higher AChR-blocking abs titres were associated with less severe MG and reduced complement system activation, indicating a potential protective mechanism for those abs. These findings suggest that AChR-blocking abs could serve as a potential biomarker for a milder disease course and highlight the need for further research to understand their role in MG pathology, which will improve strategies for clinical management and diagnosis. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  Group: Ab
  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1007/s10072-024-07889-8
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              Text: Apr2025
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              Y: 2025
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