Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Scleroderma, Esophageal

  • J. Alberto Pereira da SilvaEmail author
Reference work entry


Esophageal; Esophagus in progressive systemic sclerosis (PSP); Esophagus in systemic sclerosis (SSc); Scleroderma


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

  1. 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.CrossRefGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. Sjogren, R. W. (1994). Gastrointestinal motility disorders in scleroderma. Arthritis and Rheumatism, 37, 1265–1282.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Serviço de Reumatologia, Hospital de S. MariaLisbonPortugal