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Medical Report

Digestive System (Gut, Gastro-intestinal) Involvement in Scleroderma

By Dinesh Khanna, M.D., MSc, Assistant Professor of Medicine, Division of Immunology, University of Cincinnati, Cincinnati, Ohio, and Daniel E Furst, M.D., Carl Pearson Professor of Medicine, UCLA School of Medicine, Los Angeles, California (originally published in "Scleroderma Voice," 2004 #4)

From left: Dinesh Khanna, M.D., MSc, Assistant Professor of Medicine, Division of Immunology, University of Cincinnati, Cincinnati, Ohio, and Daniel E Furst, M.D., Carl Pearson Professor of Medicine, UCLA School of Medicine, Los Angeles, California

Editor's note: This is a long article. You can skip directly to the subheads that interest you, by clicking on these links:

• Esophagus (Food Pipe)
• Stomach
• Small Intestine (small bowel)
• Large Intestine (colon)
• Rectum
• Liver
• Summary

After the skin, the digestive system is the most commonly affected organ system in people with scleroderma, affecting about 75–90 percent of all patients. The majority of people with gut involvement experience symptoms that interfere with their day-to-day activities and quality of life.

The function of the gut is to push the food and liquid down from the mouth to the large intestine (or colon), extract and absorb nutrients, and excrete the waste in the form of stool. It does so by well-orchestrated and rhythmic motions of the gut muscles (also known as peristalsis). The primary events that cause trouble in the scleroderma gut are due to a decrease in the blood supply to the nerves, which are needed to stimulate the bowel. With decreased stimulation, there is progressive weakening of muscle strength and tone and resultant slowing and dys-coordinated motion of the gut. Virtually every gut symptom is the result of weakening of the gut muscle. The weakening starts in the esophagus (food pipe) and stomach, and works its way down to the small and large intestine.

This article will explain the reasons for the symptoms you might be experiencing from the top down, so to speak, and discuss treatments as well.

ESOPHAGUS (FOOD PIPE)

The esophagus transports food to the stomach by coordinated contractions of its muscular lining. This process is automatic and people are usually not aware of it, except when they swallow something too large, try to eat too quickly, or drink very hot or very cold liquids. They then feel the movement of the food or drink down the esophagus into the stomach, which may be an uncomfortable sensation.

The esophagus is the area where most scleroderma patients have problems, and fortunately, it has been the area where the most advances have been made in therapy. The most common symptom is heartburn (sensation of burning behind the breast bone, also known as GERD [gastro-esophageal “REFLUX” disease]), which is due to the irritation of esophagus by acid regurgitating (backing up or REFLUX) from the stomach. Reflux is caused by weakening of the gut muscle valve between the stomach and esophagus, allowing acid to “splash” back up into the esophagus. The acid, while tracking back into the food pipe, can sometimes irritate vocal cords and go into the lungs, causing hoarse voice and symptoms of asthma (wheezing and shortness of breath). Some clinical studies have even suggested that the acid going into the lungs may cause lung inflammation in scleroderma. In addition, weakening of the esophageal muscles themselves results in less efficient “milking” of the food down the esophagus, and at times food can even get temporarily “stuck” in the esophagus and patients may need to vomit to clear the esophagus. Reflux can also cause symptoms of choking, chest pain, difficulty swallowing, and acid taste in the mouth.

Diagnosis
Diagnosis is based on symptoms. Most physicians will first treat these symptoms with medications that can help decrease the acid production. If the symptoms continue, tests such as barium swallow, endoscopy, and esophageal manometry can be performed. The barium swallow requires the person to swallow a liquid, which contains barium mixed in a drink, and then take an x-ray of their esophagus and stomach. Because these organs are normally not visible on x-rays, barium temporarily coats the lining of the esophagus, stomach, and intestine, making the outline of these organs visible on the x-ray pictures. This process helps to look for the motility or gut muscle integrity and ulcers (breakdown in the lining of the gut) in the esophagus and stomach. Esophageal manometry requires a small tube be put through the nose into the esophagus. The tube is built to sense pressure, so it can measure sequential muscle contractures (the “milking” motion), and evaluate the motility and function of the esophageal muscles and the esophagus-stomach sphincter. Endoscopy requires a gastroenterologist to put a small camera at the end of a small tube (the endoscope) through your mouth into the esophagus and stomach to look for damage caused by the acid, such as ulcers and strictures (narrowing of the food pipe due to chronic acid damage).

Why do we need to treat Reflux?
The first and foremost reason to treat reflux is to relieve symptoms and improve your quality of life. (As mentioned before, acid reflux can lead to lung inflammation and even scarring of the lung tissue.) Continuous high acid production for a long period of time, when the esophagus-stomach is weak, can cause inflammation and scarring of the esophagus (also known as stricture), which can lead to Barrett’s esophagus (a potentially precancerous condition).

Periodic endoscopic examinations to look for early warning signs of cancer are generally recommended for people who have Barrett’s esophagus, an approach referred to as surveillance.

Therapy
The treatment of acid reflux requires both lifestyle changes and medications that can 1) decrease acid production; and 2) increase the motility of the gut muscle.

Lifestyle Changes
Table 1 goes over the lifestyle changes that may help significantly improve reflux symptoms.

Medications
Proton pump inhibitors (PPIs)
The PPIs are medications that suppress acid production. The goal of the treatment is to completely stop the symptoms of heartburn because continuing symptoms, even if occasional, reflect ongoing acid production. To achieve this, higher than recommended doses of PPIs are sometimes used, as described in Table 2. Another advice for people with scleroderma and reflux is to NEVER leave home without the PPIs, as missing of couple of doses can cause an increase in acid production and rebound of severe symptoms.

Certain over the counter medications, such as Zantac®, Pepcid® (known as H2 blockers) may provide temporary relief, but are not very effective in controlling the reflux symptoms. Some physicians use combinations of the PPIs and H2 blockers.

The prokinetic medications (those that improve motility of the gut muscles) can also be used if the symptoms of reflux continue even after reaching maximum doses of PPIs. (Table 2)

During the 2004 Scleroderma National Conference, many patients inquired about Zelnorm ®, a new FDA approved medication for irritable bowel syndrome (symptoms of belly pain, bloating and constipation). Zelnorm® duplicates the action of serotonin, a naturally occurring chemical in the body that helps keep the digestive system working normally. Zelnorm® coordinates the nerves, muscles, and fluid in the gut, so it starts functioning more normally. Although not FDA approved or formally studied in patients with scleroderma, Zelnorm has been used and some people say it may be helpful to stimulate the gut motility and contractility. Obviously, this medication still needs to be studied in scleroderma.

Surgery
As mentioned before, the treatment of the stricture (which is the scarring of the esophagus due to repeated acid damage) may require dilatation of the narrowing of esophagus during an endoscopy procedure. Progres-sively longer tubes are gently passed through the narrowed areas to stretch it and open it up. This helps improve the downward motion of the food. This MUST be followed by good control of the reflux symptoms otherwise the damage to the esophagus will continue and the stricture will return. The stricture, in fact, may require repeated dilations to keep it open, but the need to redilate usually decreases over time. Surgery should be the last resort as it often does not work very well. This should only be considered after a lengthy discussion with your rheumatologist. The procedure should be performed by surgeons who have experience in scleroderma.

STOMACH

Stomach symptoms in scleroderma are due to slow emptying of the food into the small intestine. The retention of food in the stomach leads to a sensation of nausea, vomiting, fullness, or bloating (also known as gastroparesis). In some people with scleroderma, the stomach can also have telangiectasia (dilated blood vessels) lining the walls of the stomach. This is also known as “watermelon stomach” due to its appearance on endoscopy. Slow and intermittent or rapid bleeding from these dilated blood vessels can cause anemia (low red blood cell count). The person may or may not have stomach symptoms and may only feel VERY tired and fatigued.

Diagnosis
Gastroparesis can be diagnosed using the barium swallow, as discussed in the esophagus section. Also, a gastric emptying study can be performed in which a person eats a very small amount of radioactive material (which is safe) with food (e.g., a sandwich or egg) followed by x-rays of their stomach. The doctors are trying to see how long it takes the food to empty into the small bowel. In scleroderma the food and radioactive material remain in the stomach longer than normal (sometimes hours) before emptying completely.

Diagnosis of “watermelon stomach,” with its characteristic appearance, can be made by an experienced gastroenterologist using endoscopy.

Treatment
The treatment of gastroparesis requires similar measures as mentioned in Table 1, and use of prokinetic medications mentioned in Table 2. The prokinetic medication should be taken one hour before the meal so the medication is “on board” when one eats. For “watermelon stomach,” use of a laser to coagulate or burn the bleeding vessels can lead to improvement in the anemia and symptoms. Patients may require multiple laser treatments before the bleeding stops completely.

SMALL INTESTINE (SMALL BOWEL)

The small bowel is the place where we gain most of the nutrition from our food. The food is pushed from the stomach into the small bowel by the rhythmic motions of the gut. Once in the small bowel, the nutrients and vitamins are extracted from the food and the waste is propelled into the large bowel and excreted as stool. The symptoms because of bowel involvement are due to lack of muscle tone, leading to stagnation of food in the small and large bowel.

In the small bowel, the symptoms are similar to gastroparesis (bloating, nausea, and vomiting), but abdominal pain may also occur. In addition, due to stagnation of food, the bacteria, which normally reside in small quantities in the small bowel, can multiply in the food causing “bacterial overgrowth syndrome.” These bacteria compete with nutrients and vitamins causing vitamin deficiencies, weight loss, and inability to gain weight. There may also be symptoms of diarrhea with foul smelling stools, which may be oily or hard to flush.
Another small bowel symptom that occurs in scleroderma is pseudo (false)-obstruction. In this condition, the bowel is not physically blocked as in true bowel obstruction but has just “had enough” and stops working! This is caused by weakening of the gut muscle. The patient complains of belly pain, distention, vomiting, and inability to “pass gas.”

Diagnosis
Bacterial overgrowth is usually diagnosed based on symptoms and treated accordingly. If the symptoms persist after treatment, a breath test can be performed. In this, person ingests sugar (lactose), which is broken down by the bacteria in the small bowel and the by-product, hydrogen, is detected in the breath. The physician should also evaluate vitamin deficiencies such as Vitamin B12, D, and folic acid

Diagnosis of pseudo-obstruction requires a plain x-ray and a CT scan of the abdomen. The CT scan detects whether the symptoms are due to a tired “gut,” one that needs rest, or there is something obstructing the small bowel.

Treatment
Bacterial overgrowth is treated using antibiotics to reduce the overgrowth of bacteria in the small bowel. This is achieved by using antibiotics for 10–14 days at a time (Table 2). Usually a person notices improvement in the symptoms after a course of treatment and may notice a slow weight gain. Very often, the treatment has to be repeated every month or so to keep the bacteria level low. Occasionally treatment must be continuous and some physicians use different antibiotics every month to prevent the bacteria from getting resistant. Pro motility agents (Table 2) can also help. Patients may also obtain symptomatic relief in the bacterial overgrowth syndrome by regular use of yogurt containing bowel friendly bacteria. StonyfieldFarm® yogurt was the favorite among the participants of this year’s National Scleroderma Conference, although any yogurt should work.

Sandostatin® (octereotide) has been successfully used during attacks of pseudo-obstruction, and if attacks are frequent, can sometimes be used on a regular basis to prevent any further attacks. Once treated, a person should take prescribed multivitamins to prevent or treat vitamin deficiencies.

Pseudo-obstruction requires hospitalization and complete rest of the small bowel. The person is given nutrients through the veins along with pro-motility agents. Venting is also performed when needed. “Venting” means to put a small surgical tube into the jejunum (small intestine) to aspirate out the air. This keeps the patient from becoming severely distended while in treatment. Total parenteral nutrition (TPN) [high energy liquid diet through the veins] may be prescribed by your physician to give your bowel a prolonged rest and help provide you with essential nutrition and energy. In many patients, the bowel slowly recovers after 2–3 months, and people can go back to eating regular food.

LARGE INTESTINE (COLON)

The main function of the large bowel is to reabsorb water and salts that have been secreted by the rest of the gut. This helps the formation of stools and helps to move the waste along. In scleroderma, there is, as is the case with the rest of the gut, weakening of the gut muscles and impaired motility. This can lead to constipation. Constipation means different things to different people. For many people, it simply means infrequent stools. Medically speaking, constipation usually is defined as fewer than three bowel movements per week. Severe constipation is defined as less than one bowel movement per week. This is usually associated with pain and a feeling of incomplete emptying of the bowel. Also, one may get wide mouthed diverticula (large pouches) in the colon. These do not ordinarily cause any symptoms. However, on rare occasions, the stool can get impacted in them causing inflammation of the diverticula, called diverticulitis (this is similar to, but not exactly the same as the common diverticulitis that you often hear about). Diverticulitis usually presents with belly pain and fever. People can also get diarrhea due to impacted stool.

Diagnosis
Diagnosis usually starts with symptoms leading to the colon. A barium study can help look at the large bowel, but a word of caution: the impacted barium may lead to perforation or damage to the bowel. Therefore, after any barium study, the person should receive a laxative such as Colace® or Senna® to remove the barium from the bowel.

Treatment
Treatment for constipation requires laxatives that stimulate the nerve endings in the gut wall, which make the muscles in the intestine contract with more force. A high fiber diet without a laxative is not helpful as it just adds to the amount of waste the bowel is struggling with. There is debate whether the chronic use of laxatives such as Senna® can somewhat worsen constipation over time; however, there are no studies to definitely show that. The person can take a laxative every other day to maintain a healthy bowel regimen.

RECTUM

Stool incontinence (symptom of accidentally soiling the underwear before being able to get to a bathroom) occurs in up to a third of people with scleroderma. This is due to the weakening of the rectal muscle and poor control the over rectal sphincter. Biofeedback therapy may be helpful by improving voluntary squeezing of the rectal muscle. Major scleroderma centers offer classes to teach people this technique. Surgery by an experienced surgeon might also be helpful.

LIVER

The liver plays an important role in the detoxification of drugs in our body. About 10% of scleroderma patients may have liver involvement, known as primary biliary cirrhosis. If this very uncommon complication occurs, it usually occurs 10–15 years after the onset of scleroderma. Symptoms usually consist of itchy skin and fatigue. The diagnosis can be made by blood tests for anti-mitochondrial and anti-smooth muscle antibody and the treatment consists of doses of ursodeoxycholic acid.

SUMMARY

In summary, scleroderma frequently affects the gut. The symptoms can be distressing and can cause impairment of a person’s quality of life as well as other complications. Dependable laboratory and radiographic tests are available to find the location and extent of involvement. Appropriate treatments are often very effective.