Definition

Scleroderma (or systemic sclerosis) is a rare disease of the connective tissues of the whole body. It can be localized to the skin or can affect multiple organs in the body including the gastrointestinal system. It starts with deposition of collagen in the vessels, muscles, skin, lungs, kidneys and the gastrointestinal system, affecting their normal function.

Causes

While the causes of scleroderma are not clear, it has been suggested that exposure to certain chemicals can play a role. Scleroderma is more common in women than men and usually affects people in their 30s to 50s.

Symptoms

Esophageal scleroderma can present with weight loss, dysphagia (difficulty swallowing), bloating, regurgitation and severe heartburn. Dysphagia, heartburn, and regurgitation are usually due to reflux and dysmotility of the esophagus. Esophageal strictures and Barrett’s esophagus can also be associated with esophageal scleroderma. The symptoms usually progress slowly with time. 

Diagnosis

Blood tests to check for autoimmune markers are a start to diagnose scleroderma. The evaluation of esophageal scleroderma starts with an upper endoscopy to evaluate for possible strictures and other causes of dysphagia. High-resolution manometry is important to diagnose esophageal dysmotility and a videoesophagram is also a sensitive test to diagnose scleroderma.

Treating scleroderma starts with steroids and immunomodulators that are usually prescribed by rheumatologists (physicians that specialize in the treatment of connective tissue disorders). In regard to the esophageal symptoms, treatment is usually aimed to relieve symptoms and prevent complications. To control gastroesophageal reflux disease we use acid suppressants like H2–blockers (e.g., ranitidine and famotidine) and proton pump inhibitors (omeprazole, lansoprazole, pantoprazole and their variants) to help control acid reflux.

Prokinetics such as metoclopramide can be used to improve poor motility across the esophagus that is associated with scleroderma. In addition, aspiration precautions are necessary to prevent some pulmonary-associated complications in patients with severe esophageal dysmotility. Complications such as candida esophagitis can be treated with antifungal medications.

In a selected group of patients, the esophagus can be connected to a part of the small bowel to prevent the reflux of acid and bile (non-acidic fluid) from the stomach into the esophagus. The procedure is similar to gastric bypass and can be performed via a minimally invasive approach.