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Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies

Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportiv...

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Autores principales: Mendoza, Fabian A., DiMarino, Anthony, Cohen, Sidney, Adkins, Christopher, Abdelbaki, Shady, Rattan, Satish, Cao, Christopher, Denuna-Rivera, Susie, Jimenez, Sergio A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699580/
https://www.ncbi.nlm.nih.gov/pubmed/36431141
http://dx.doi.org/10.3390/jcm11226665
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author Mendoza, Fabian A.
DiMarino, Anthony
Cohen, Sidney
Adkins, Christopher
Abdelbaki, Shady
Rattan, Satish
Cao, Christopher
Denuna-Rivera, Susie
Jimenez, Sergio A.
author_facet Mendoza, Fabian A.
DiMarino, Anthony
Cohen, Sidney
Adkins, Christopher
Abdelbaki, Shady
Rattan, Satish
Cao, Christopher
Denuna-Rivera, Susie
Jimenez, Sergio A.
author_sort Mendoza, Fabian A.
collection PubMed
description Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportive, although severe cases are usually refractory to conventional management. Recent studies have shown that the abnormal production of functional autoantibodies such as anti-cholinergic muscarinic receptor III antibodies may participate in the pathogenesis of SSc-associated gastrointestinal dysmotility and may provide a novel target for therapeutic intervention. We describe two patients with severe and rapid onset of SSc-associated severe swallowing dysfunction and esophageal dysmotility who had failed standard of care therapy, requiring complete enteral and parenteral nutrition. Both patients were positive for the presence of circulating antimuscarinic III receptor antibodies. They were treated with IVIG at a dose of 2 g/Kg/month divided in two consecutive days, for six months. Following IVIG therapy, both patients markedly improved their symptoms as shown by a reduction in their UCLA2.0 score, and achieved an improvement of esophageal motility documented radiologically. Both patients resumed oral feeding and had their feeding tubes removed within the treatment period. None of the patients developed severe adverse events attributable to IVIG, except for low-grade fever during IVIG infusion in one of the cases. These results provide support for the role of functional autoantibodies in the development of SSc-associated gastrointestinal dysfunction.
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spelling pubmed-96995802022-11-26 Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies Mendoza, Fabian A. DiMarino, Anthony Cohen, Sidney Adkins, Christopher Abdelbaki, Shady Rattan, Satish Cao, Christopher Denuna-Rivera, Susie Jimenez, Sergio A. J Clin Med Case Report Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportive, although severe cases are usually refractory to conventional management. Recent studies have shown that the abnormal production of functional autoantibodies such as anti-cholinergic muscarinic receptor III antibodies may participate in the pathogenesis of SSc-associated gastrointestinal dysmotility and may provide a novel target for therapeutic intervention. We describe two patients with severe and rapid onset of SSc-associated severe swallowing dysfunction and esophageal dysmotility who had failed standard of care therapy, requiring complete enteral and parenteral nutrition. Both patients were positive for the presence of circulating antimuscarinic III receptor antibodies. They were treated with IVIG at a dose of 2 g/Kg/month divided in two consecutive days, for six months. Following IVIG therapy, both patients markedly improved their symptoms as shown by a reduction in their UCLA2.0 score, and achieved an improvement of esophageal motility documented radiologically. Both patients resumed oral feeding and had their feeding tubes removed within the treatment period. None of the patients developed severe adverse events attributable to IVIG, except for low-grade fever during IVIG infusion in one of the cases. These results provide support for the role of functional autoantibodies in the development of SSc-associated gastrointestinal dysfunction. MDPI 2022-11-10 /pmc/articles/PMC9699580/ /pubmed/36431141 http://dx.doi.org/10.3390/jcm11226665 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Mendoza, Fabian A.
DiMarino, Anthony
Cohen, Sidney
Adkins, Christopher
Abdelbaki, Shady
Rattan, Satish
Cao, Christopher
Denuna-Rivera, Susie
Jimenez, Sergio A.
Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title_full Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title_fullStr Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title_full_unstemmed Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title_short Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies
title_sort treatment of severe swallowing dysfunction in systemic sclerosis with ivig: role of antimuscarinic antibodies
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699580/
https://www.ncbi.nlm.nih.gov/pubmed/36431141
http://dx.doi.org/10.3390/jcm11226665
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