Introduction
Patients with scleroderma esophagus lack peristalsis (muscle contraction that aids in swallowing), resulting in dysphagia. There is a ring of muscles at the esophagogastric junction (the junction between the esophagus and the stomach) called the lower esophageal sphincter (LES). Scleroderma patients frequently have a weak LES, making them more susceptible to gastroesophageal reflux disease (GERD), a condition in which gastric juices are regurgitated back into the esophagus.
What Is Scleroderma Esophagus?
Scleroderma, or systemic sclerosis, is a connective tissue disorder. It is usually expressed as fibrosis and thickening of the skin. Visceral involvements may include the heart, liver, kidney, and gastrointestinal tract. The esophagus is this condition's most commonly affected organ of the digestive system. This can also affect the patient's quality of life. Scleroderma is usually seen as fibrosis and thickening of the skin. Visceral involvements may include the heart, liver, kidney, and gastrointestinal tract. The esophagus is the most commonly affected organ in the digestive system under this condition. This can also affect the patient's quality of life. After the changes in the skin and the Raynaud phenomenon (a condition where the fingers lack proper blood supply), gastrointestinal changes are the next common manifestations of systemic sclerosis.
There is a ring of muscles at the esophagogastric junction (the junction between the esophagus and the stomach) called the lower esophageal sphincter (LES). Scleroderma patients frequently have a weak LES, making them more susceptible to gastroesophageal reflux disease (GERD), a condition in which gastric juices are regurgitated back into the esophagus.
What Is Scleroderma?
Scleroderma is a group of connective tissue disorders generally classified as autoimmune rheumatic disorders. The word 'scleroderma' is derived from two Greek words: sclero (hard) and derma (skin). This is the most common feature of patients with scleroderma, thickening of the skin. The exact pathophysiology is complex and still needs to be fully understood. Scleroderma is caused due to excess deposition of collagen, a protein found in the connective tissue) in the skin and other smooth muscles.
How Does Scleroderma Affect the Esophagus?
The fibrosing and thickening in the skin are also visible in the smooth muscles throughout the body and the gastrointestinal tract, affecting the esophagus. It causes damage to normal tissue, leading to fibrosis of the healthy tissue. In addition, scleroderma makes the esophageal muscle weak, causes strictures, or narrows it. This makes it difficult to transport food and liquids down the esophagus, a condition that the physicians call motility disturbance.
The condition can cause two problems that might interfere with the patient's quality of life:
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The passage of food is very slow, making the swallowing process hard and making the patient feel full and bloated.
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The LES muscle does not close properly, causing acid regurgitation. A rheumatologist usually treats scleroderma. But the gastrointestinal issues involved might require a gastroenterologist to be involved as well.
What Are the Symptoms of Scleroderma?
Scleroderma affects the internal organs by replacing the muscles and nerves with collagen, thereby causing them to thicken. This causes muscle stiffness and a loss of movement coordination. The presentation of symptoms is representative of the affected gastrointestinal organ. If the anal sphincter is involved, it can cause fecal incontinence (an inability to control bowel movements). If the colon is affected, it might result in diarrhea or constipation. Motor dysfunction of the small intestine can cause undigested matter to accumulate in the small intestine and increase bacterial growth in the small intestine. This results in bloating, gassiness, and diarrhea. These are described as symptoms of internal obstruction without mechanical obstruction. Scleroderma of the stomach causes gastroparesis (the transport of food from the stomach to the small intestine becomes slow, also called delayed emptying). The signs of gastroparesis include a comfortable feeling of fullness, nausea, vomiting, and abdominal pain.
Esophageal involvement is the most common feature of scleroderma. Scleroderma patients have a weak LES, making them more susceptible to gastroesophageal reflux disease (GERD). If left untreated, GERD can progress to esophagitis, esophageal ulceration, esophageal stricture, or Barrett's esophagus, which can be a precursor to esophageal cancer. Barrett's esophagus is a condition where the esophageal lining is damaged due to frequent exposure to gastric acids.
In a normal person, swallowing occurs due to a set of simultaneous movements in the esophagus called peristalsis. In patients with scleroderma, the esophagus shows peristalsis, where the peristaltic movement is absent. When this happens, food fails to move down, causing dysphagia or a sensation of food stuck in the chest. This also causes an increase in GERD. In addition, this prolonged acid regurgitation can lead to esophageal ulcerations.
How Is Scleroderma Esophagus Diagnosed?
Many diagnostic tools are used to diagnose scleroderma of the esophagus. Alarming symptoms like dysphagia, odynophagia (painful swallowing), nausea, vomiting, anemia, weight loss, and blood in the stool might require an endoscope. Patients with endoscopy and no underlying pathology may require esophageal manometry. Manometry helps the doctor measure the strength of the muscle while swallowing a liquid, a viscous liquid, or a solid. This method can also identify weaknesses of the LES, defects in the peristaltic movements, and, in some cases, pharyngeal dysfunctions.
How Is Scleroderma Esophagus Treated?
Treating a scleroderma esophagus can be challenging. Changes in diet can, to an extent, help relieve the symptoms. Frequent small meals can help with delayed emptying. Having dinner a few hours before sleep can reduce the contents of the stomach, reducing regurgitation and aspiration while asleep. Patients with scleroderma esophagitis should avoid or limit their intake of caffeine and chocolate. These contain phosphodiester inhibitors, which relax the gastrointestinal muscles and weaken esophageal contractions, thereby delaying gastric emptying. Alcohol can also delay emptying. Sleeping with the head elevated 4 to 6 inches has been found to relieve regurgitation symptoms.
Medication includes medicines that reduce gastric acid production, like proton pump inhibitors and H2 antagonists, and medicines that improve gastric emptying, like dopamine antagonists and 5-HT4 antagonists. Surgical approaches may be required when GERD symptoms worsen despite the medications or the condition worsens, like affecting liver function. Two surgical approaches are commonly used:
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Toupet's Fundoplication: This does not wrap completely around the esophagus; posteriorly, the fundus is wrapped only two-thirds of the way at the bottom of the esophagus. This creates a valve-like structure that allows you to release gas through burps or vomit if necessary.
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Roux-en-Y Gastric Bypass: Also called gastric bypass, it creates a small pouch from the stomach, connecting it to the small intestine.
Conclusion:
Scleroderma esophagus is very common in systemic sclerosis patients, which deteriorates the quality of their lives. Proton pump inhibitors are the first line of treatment. Surgical therapy should be limited to patients who do not respond or respond poorly to the medications and should be well documented.
