Synonyms
Esophageal; Esophagus in progressive systemic sclerosis (PSP); Esophagus in systemic sclerosis (SSc); Scleroderma
Definition
Esophageal disease in scleroderma is the result of dysmotility and lower esophageal sphincter dysfunction.
Both neurogenic impairment and microvascular insufficiency lead to smooth muscular atrophy and fibrosis. Endothelial activation is followed by the production of autoantibodies. Autoantibodies against enteric neurons and anti-muscarinic antibodies have been described in some patients.
Dysmotility is a consequence of smooth muscle involvement in the distal two-thirds of the esophagus. Esophageal sphincter dysfunction with reduced pressure leads to gastroesophageal reflux.
Involvement of the upper esophagus is the result of inflammation of striated muscle and should raise the possibility of other diagnosis (overlap syndromes or dermatomyositis).
Symptoms consist of retrosternal pain, dysphagia, heartburn, and regurgitation. Delayed gastric emptying,...
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References and Further Reading
Clarke, J. O., & Hirano, I. (2012). Upper gastrointestinal tract. In J. Varga et al. (Eds.), Scleroderma: From pathogenesis to comprehensive management (pp. 471–484). New York: Springer.
Doma, S., Wo, J. M., & Parkman, H. P. (2012). Esophageal involvement in systemic diseases. In J. E. Richter & D. O. Castell (Eds.), The esophagus (pp. 367–382). Chichester: Wiley-Blackwell.
Sjogren, R. W. (1994). Gastrointestinal motility disorders in scleroderma. Arthritis and Rheumatism, 37, 1265–1282.
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da Silva, J.A.P. (2017). Scleroderma, Esophageal. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1680
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1680
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