In scleroderma, the immune system damages healthy tissue like collagen and replaces it with scar tissue, causing thickening and tightening of the skin as well as damage to other organs and systems.
Localized scleroderma is generally used to designate disease that is confined to the skin, while systemic sclerosis and scleroderma are generally used to indicate disease that is affecting organs throughout the body, but we will use them somewhat interchangeably in this article.
Learn more general information here about scleroderma and systemic sclerosis.
Systemic sclerosis causes all-over inflammation and thickening and tightening of connective tissue, which can cause scarring and damage in different areas of the body, including the lungs, heart, kidneys, and the gastrointestinal tract, where it can lead to very obvious symptoms like acid reflux and pain and difficulty swallowing.
Gastrointestinal (GI) involvement in scleroderma and systemic sclerosis is very common; it is thought to affect some 90 percent of patients, with symptoms ranging from mild to severe. GI symptoms are much more common in people with systemic disease compared with localized disease.
The esophagus — the tube that connects the mouth to the stomach, carrying food and liquids to be digested — is the most commonly affected part of the GI tract in systemic sclerosis.
This is sometimes referred to as esophageal scleroderma.
Here, learn more about what causes esophageal scleroderma, symptoms to watch for, and how it is treated and managed.
How Scleroderma Affects the Esophagus
The same scarring and thickening that causes outward skin changes can also affect smooth muscle tissue in organs throughout the body. In the GI tract, scleroderma can affect the smooth muscle of the esophagus, damaging healthy tissue and replacing it with scar tissue.
Scleroderma causes strictures, or narrowing, of the esophagus and makes the muscle tissue weaker. This can cause what doctors refer to as “motility disturbance” — or trouble with food and liquid being able to travel down the esophagus. This leads to two main issues that affect patients’ quality of life:
- Food travels more slowly, which can make swallowing difficult and make people feel full and bloated
- The sphincter muscle between the esophagus and the stomach (which helps prevent food from traveling backward into the esophagus after it has entered the stomach) doesn’t close fully, causing gastroesophageal reflux, also called acid reflux or heartburn
“Esophageal involvement in scleroderma is incredibly common, but some of that depends on how it is defined and how aggressively it is searched for,” says Robert Spiera, MD, rheumatologist and director of the Vasculitis and Scleroderma Program at the Hospital for Special Surgery in New York City.
While scleroderma and systemic sclerosis are usually treated by a rheumatologist, people who have worsening GI involvement usually need additional treatment from a gastroenterologist.
Symptoms of Scleroderma in the Esophagus
When you eat, foods travels from your mouth, to your throat, and into the stomach through a tube called the esophagus. Normally, a valve at the bottom of the esophagus acts as a one-way gate that opens to let food enter the stomach, then closes to prevent food from coming back up.
When you have systemic sclerosis, the gate doesn’t close properly, so partially digested food that has entered the stomach can back up into the esophagus. This known as gastroesophageal reflux disease, or GERD.
GERD may cause a burning sensation (heartburn) in your chest, throat, or mouth as food and acid return into the esophagus. Acid from the stomach, which is used in digestion, can injure the lining of the lower portion of the esophagus, causing further scarring and narrowing of the tube.
Other symptoms may include:
- Difficulty swallowing
- Pain when swallowing
- Feeling like food is stuck in your esophagus
- Choking on food
- Chest pain
- Hoarseness or lost voice
About half of patients with scleroderma have GERD or dysphagia (difficulty swallowing), says Dr. Spiera.
“People will say it feels like something is caught in their throat,” says Kenneth Beer, MD, a dermatologist in West Palm Beach, Florida, who is affiliated with Good Samaritan Medical Center in West Palm Beach.
When scleroderma affects other parts of the GI tract, such as the small intestine, large intestine, or the anal sphincter, it can cause additional symptoms such as diarrhea, constipation, bloating and gassiness, fecal incontinence, and gastroparesis, which is when it takes food a long time to leave the GI tract during digestion. Gastroparesis symptoms including feeling full quickly, nausea, vomiting, and abdominal pain.
How Esophageal Scleroderma Is Diagnosed
Doctors can run different tests doctors to assess how scleroderma is affecting the esophagus and gastrointestinal tract. Generally, doctors perform these tests only in patients who are complaining of GI-related symptoms, like heartburn or difficulty swallowing.
This procedure uses a thin, flexible tube with a tiny camera on the end. It’s inserted through the mouth to look at the upper digestive system. This test can reveal changes to the esophagus such as inflammation, infection, Barrett’s esophagus (changes to the lining of the esophagus that can increase the risk of esophageal cancer), and strictures (narrowing of the esophagus).
This test is used to see whether the lower esophageal sphincter muscle is working properly. Is it helping to keep food in the stomach (where it belongs) or allowing food to backflow into the esophagus? A small flexible tube is passed through the nose, down the esophagus, and into the stomach. During the test, people may swallow liquid (including water and thicker liquid) or food and the doctor will observe how well the esophageal muscles and sphincter muscle are working during digestion.
This special kind of X-ray may reveal more information about symptoms such as difficulty swallowing, gastroesophageal reflux disease, and other GI issues. It uses barium, a chemical that makes X-rays easier to see, and can reveal the size and shape of your esophagus and how well you swallow.
Esophageal pH testing
This test measures the pH of your esophagus (or how acidic it is) to see whether you have gastroesophageal reflux disease or how it is responding to treatment. A small, thin tube with a little device on the end that senses acid is passed through your nose, down your esophagus, and kept slightly above the sphincter muscle between the esophagus and the stomach. The tube is attached to your nose with clear tape and attached to a recorder that you wear (such as on your belt). You will follow your normal eating habits for 24 hours while the device records what’s happening.
Even in patients with symptoms suggesting esophageal involvement, not all of these tests will necessarily be ordered. Your doctor may also recommend other tests as needed, including more advanced ones to look at gastric emptying and motility.
Complications and Risks from Scleroderma in the Esophagus
Gastroesophageal reflux disease — when contents from the stomach flow backward into the esophagus, often causing symptoms like heartburn — is not just an annoying digestive issue. Left untreated, it can cause inflammation of the esophagus and further contribute to scarring and narrowing of the esophagus that is already happening from scleroderma-related immune system damage.
Over time, this can lead to a condition called Barrett’s esophagus, which is when the tissue lining the esophagus changes and becomes more like the tissue lining the intestines. This can increase the risk of developing esophageal cancer.
Once you are diagnosed with Barrett’s esophagus, your doctor will be checking regularly for signs of precancerous changes, which can be treated and removed. Most people with Barrett’s esophagus will not develop esophageal cancer.
Aspiration and Lung Issues
In people with severe esophageal scleroderma, having a combination of GERD and gastroparesis (slow/delayed digestion) can lead to the aspiration of stomach contents into the airway (throat). That can cause cough and respiratory issues, including pneumonia or worsening lung function.
An esophageal stricture is a narrowing of the esophagus. In people with scleroderma, the most common cause of an esophageal stricture is long-standing GERD. The main symptom is difficulty swallowing, known as dysphagia. You may feel like food is sticking or having a hard time going through your chest, upper abdomen, or throat. You may at first have a tough time swallowing solids or pills. It may be hard to swallow liquids as the narrowing worsens.
Treating Scleroderma in the Esophagus
Managing esophageal scleroderma is generally about addressing troublesome symptoms like heartburn and difficulty swallowing. Treating GERD in patients with scleroderma is usually similar to treating GERD in patients without it, although symptoms in esophageal scleroderma are usually more severe.
This can be done through a combination of medications and lifestyle changes. Surgical procedures may be recommended in more advanced cases.
Treating GERD and other GI issues begins with lifestyle changes, but these are often not enough to treat symptoms in esophageal scleroderma. They include:
Eat smaller and more frequent meals throughout the day. “Since your motility is diminished, it is hard for the GI tract to handle a larger volume load,” says Dr. Spiera. “You don’t, however, want to restrict total calorie consumption.” Leave some room in your stomach and don’t eat to the point that you’re completely full, suggests Ali Ajam, MD, a rheumatologist at The Ohio State University Wexner Medical Center in Columbus.
Stay in an upright position after meals to help keep down food. Gravity helps keep food and acid in the stomach.
Don’t eat within a few hours of bedtime. This can cause heartburn and stomach pain at night, making it difficult to sleep. “Since gastric emptying can be delayed, eating earlier can be helpful as more of the food can move down the GI tract and enter the small intestine or beyond by the time you go to bed,” says Dr. Spiera. “That would minimize the likelihood of reflux of contents coming up from the stomach.”
Elevate the head of your bed your bed a few inches. “This helps use gravity to assist food moving along the GI tract, in particular the esophagus,” says Dr. Spiera. You can use blocks, bricks, or plastic bed risers on the floor, or purchase a wedge that goes under the mattress or a wedge-shaped pillow to keep your head slightly elevated while you sleep.
Avoid tight-fitting clothing. Many patients with scleroderma get bloating, says Dr. Spiera. Diminished motility in the intestine can lead to bacterial overgrowth that results in bloating as well as gassiness and cramping. “Looser-fitting clothing might be less likely to exacerbate that or contribute to the bloating,” he says.
Avoid foods that make heartburn worse. Certain foods may make heartburn worse, although this can be very individualized. Consider keeping a food diary to track which foods may be a problem for you. Generally, foods that may exacerbate reflux and heartburn include:
- Caffeine (including coffee, tea, and dark sodas)
- Fried and high-fat foods
- Citrus fruit
- Tomatoes and tomato sauce
- Spicy foods
- Carbonated drinks
Eat slowly and chew thoroughly. Abnormally slow movement of foods and narrowing of the esophagus can make it difficult to swallow. Consume soft foods and avoid ones that stick in the throat such as pieces of meat, large pieces of bread, and other bulky foods.
Medication for Reflux
Antacids, especially in liquid form, can help neutralize stomach acid and reduce heartburn. People with heartburn from esophageal scleroderma may be prescribed a kind of medication called proton pump inhibitors (PPIs), which include lansoprazole (Prevacid) or pantoprazole (Protonix) or a different kind of medication called H2 receptor blockers, which include esomeprazole (Nexium), ranitidine (Zantac), or famotidine (Pepcid). They each work differently: PPIs prevent the acid pump in the stomach from secreting stomach acid. H2 receptor blockers block histamine, a chemical that promotes acid production in the stomach.
Medication for Slow Digestion
Medications that treat motility issues stimulate muscular activity in the GI tract to help keep digestion humming. These include metoclopramide (Reglan) and erythromycin (Eryc, E.E.S.).
For severe cases, other medications may be available with restricted access/under your doctor’s supervision because of an increased risk of serious side effects.
Surgery and Procedures for Esophageal Scleroderma
People with severe narrowing of the esophagus may benefit from a procedure in which the esophagus is dilated (expanded), which can make swallowing easier. Called endoscopic dilation, the procedure entails stretching the muscles of the esophagus using a balloon that is inserted and inflated. This procedure may be done periodically.
Severe GERD that is not responding to medication may be treated with different types of surgical procedures, though these have not been as well-studied in people with esophageal scleroderma and should be used with caution.
Get Involved in Scleroderma Research
If you are diagnosed with scleroderma, arthritis, or another musculoskeletal condition, we encourage you to participate in future studies by joining CreakyJoints’ patient research registry, ArthritisPower. ArthritisPower is the first-ever patient-led, patient-centered research registry for joint, bone, and inflammatory skin conditions. Learn more and sign up here.
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Interview with Ali Ajam, MD, a rheumatologist at The Ohio State University Wexner Medical Center in Columbus.
Interview with Kenneth Beer, MD, a dermatologist in West Palm Beach, Florida, who is affiliated with Good Samaritan Medical Center in West Palm Beach.
Interview with Robert Spiera, MD, rheumatologist and director of the Vasculitis and Scleroderma Program at the Hospital for Special Surgery in New York City.
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