Scleroderma usually and most of the time effects the esophagus. Almost 90 percent of patients with scleroderma have some kind of motility disorder with regards to esophagus.
Decreased esophageal peristalsis as the smooth muscle of esophagus gets fibrosed (hardens) and becomes wasted (atrophy). The lower esophageal sphincter (LES) becomes useless as it neither contracts or relaxes, basically it works as a open tube and act as an open connection between stomach and esophagus. Long standing GERD can result in barret’s esophagus which in turn lead to Adenocarcinoma of Esophagus which is a type of esophageal cancer.
Fibrosis and atrophy of esophageal smooth muscle due to scleroderma results in following signs and symptoms:
- Difficulty in swallowing as the peristaltic movement is lost due to sclerosis and fibrosis of esophageal smooth muscle
- Reflux of gastric acid from stomach to esophagus also known as Gastro Esophageal Reflux Disease (GERD)
- If a patients has past history of scleroderma and comes to visit hospital with complaint of dysphagia or GERD, the above mentioned pathogenesis is the most likely cause.
Diagnosis:
The most accurate method of diagnosing esophageal disorder in scleroderma is by motility studies like esophageal manometry. Barium studies are not necessary.
Treatment:
Treatment of underlying cause, in this case scleroderma is necessary, though the esophageal discomfort can be relieved by Proton Pump Inhibitors that will cause relief in GERD and with some pro motility agents, that will improve the contraction and relaxation of esophageal smooth muscle. Drugs like Metoclopromide have pro motility action and known to help esophageal motility disorder in scleroderma.