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[Achalasia in circumscribed scleroderma].

I Gockel1, K Bork, V F Eckardt

  • 1Klinik für Allgemein- und Abdominalchirurgie, Johannes Gutenberg-Universität Mainz. gockel@ach.klinik.uni-mainz.de

Deutsche Medizinische Wochenschrift (1946)
|August 12, 2006
PubMed
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This case report details a patient with circumscribed scleroderma who developed achalasia. Surgical intervention for achalasia provided symptom relief, suggesting a potential link between these conditions.

Area of Science:

  • Gastroenterology
  • Rheumatology
  • Autoimmune Diseases

Background:

  • Circumscribed scleroderma is a rare autoimmune condition affecting connective tissues.
  • Achalasia is a primary esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter.
  • The co-occurrence of these conditions is exceptionally rare and not previously documented.

Observation:

  • A 38-year-old male with a 12-year history of circumscribed scleroderma presented with severe dysphagia, diagnosed as achalasia.
  • The patient experienced significant weight loss and required a percutaneous endoscopic gastrostomy (PEG) due to intractable symptoms.
  • Esophageal manometry and barium studies confirmed achalasia, with endoscopic findings of a dilated esophagus and difficult passage through the cardia.

Findings:

Related Experiment Videos

  • The patient underwent an extramucosal Heller myotomy with Dor's procedure for achalasia.
  • Postoperatively, the patient experienced complete resolution of dysphagia, tolerating both solid and liquid food without difficulty.
  • The case highlights a potential, previously undescribed association between circumscribed scleroderma and achalasia.

Implications:

  • This association may suggest a common autoimmune etiology or shared pathogenetic mechanisms, potentially involving fibrosis.
  • Further research is warranted to explore the potential link between autoimmune conditions like scleroderma and esophageal motility disorders.
  • Understanding this association could lead to improved diagnostic and therapeutic strategies for patients with overlapping autoimmune and gastrointestinal conditions.