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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| Format: | Article |
| Language: | English |
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MDPI AG
2022-11-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/22/6665 |
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| author | Fabian A. Mendoza Anthony DiMarino Sidney Cohen Christopher Adkins Shady Abdelbaki Satish Rattan Christopher Cao Susie Denuna-Rivera Sergio A. Jimenez |
| author_facet | Fabian A. Mendoza Anthony DiMarino Sidney Cohen Christopher Adkins Shady Abdelbaki Satish Rattan Christopher Cao Susie Denuna-Rivera Sergio A. Jimenez |
| author_sort | Fabian A. Mendoza |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T18:14:58Z |
| format | Article |
| id | doaj.art-a834d4c2f5a04e32bf8880bd6ba8901e |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T18:14:58Z |
| publishDate | 2022-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-a834d4c2f5a04e32bf8880bd6ba8901e2023-11-24T08:48:03ZengMDPI AGJournal of Clinical Medicine2077-03832022-11-011122666510.3390/jcm11226665Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic AntibodiesFabian A. Mendoza0Anthony DiMarino1Sidney Cohen2Christopher Adkins3Shady Abdelbaki4Satish Rattan5Christopher Cao6Susie Denuna-Rivera7Sergio A. Jimenez8Department of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USADepartment of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USADivision of Gastroenterology, Department of Medicine, Thomas Jefferson University, Philadelphia, PA 19107, USAJefferson Institute of Molecular Medicine and Scleroderma Center, Philadelphia, PA 19107, USAOropharyngeal 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.https://www.mdpi.com/2077-0383/11/22/6665systemic sclerosisIVIGautoantibodiesesophageal dysmotilitytreatment |
| spellingShingle | Fabian A. Mendoza Anthony DiMarino Sidney Cohen Christopher Adkins Shady Abdelbaki Satish Rattan Christopher Cao Susie Denuna-Rivera Sergio A. Jimenez Treatment of Severe Swallowing Dysfunction in Systemic Sclerosis with IVIG: Role of Antimuscarinic Antibodies Journal of Clinical Medicine systemic sclerosis IVIG autoantibodies esophageal dysmotility treatment |
| 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 | systemic sclerosis IVIG autoantibodies esophageal dysmotility treatment |
| url | https://www.mdpi.com/2077-0383/11/22/6665 |
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