People with systemic scleroderma may develop abnormalities of the digestive system and gastrointestinal tract from the mouth to the anal canal. The overproduction of collagen typical of scleroderma can cause thickening and fibrosis (or scarring) of the tissues. This can result in weakened muscles, and lead to the abnormally slow movement of food (dysmotility) in the digestive process.
Food travels from the mouth and throat into the stomach through a tube called the esophagus. Normally, the lower esophageal sphincter, or valve, acts as a gate, which opens to allow food to enter the stomach and then closes promptly to prevent food from coming back up. In systemic scleroderma, the gate does not close properly and the result is a backwash of acid and a burning sensation (heartburn) as food and acid return into the esophagus. The acid may also injure the lining of the lower portion of the esophagus, causing scarring and a narrowing (stricture) of the tube.
Acid production can be reduced, and the problems of acid reflux and heartburn helped, by avoiding alcohol, greasy or fatty foods, spicy foods, chocolate, tobacco and caffeine. Antacids (particularly in liquid form) can help neutralize acids and reduce heartburn. Some antacids cause constipation while others cause diarrhea. Consult your physician or pharmacist when choosing over-the-counter products. Your doctor may prescribe antacid medications such as proton pump inhibitors or H-2 blockers to decrease acid production in the stomach. The physician also may prescribe a drug such as metoclopramide, which promotes muscular activity and causes the esophagus to work better. The force of gravity helps to keep food and acid in the stomach; therefore, an upright position after meals is helpful. Other common-sense measures to prevent acid from coming up into the esophagus include eating smaller and more frequent meals, not eating for several hours before bedtime, and elevating the head of the bed six-to-eight inches with wooden blocks. Being overweight is harmful, and you should avoid wearing girdles or other tight-fitting garments.
Abnormally slow movement of food and narrowing of the esophagus may cause swallowing difficulties. Eating slowly and chewing thoroughly are important. Swallowing and digesting are made easier by eating softer foods (many foods can be prepared in a blender) and avoiding foods, which tend to stick in the throat. If the esophagus has narrowed significantly, the physician may need to dilate the esophagus periodically to permit easier swallowing.
In systemic scleroderma, there can be damage to the muscles of the small bowel (small intestine). The weakened muscles do not work effectively to push food through the bowel. Simply put, things sit rather than move well. One consequence can be an overgrowth of bacteria, leading to diarrhea. There also may be a bloated, distended feeling and some pain if the bowel is stretched. Another effect is that the nutrients of food remain in the bowel instead of being absorbed into the body. This condition is called malabsorption, and it may lead to weight loss and stool abnormalities.
For diarrhea or malabsorption, the physician may prescribe an antibiotic, or supplementary fat-soluble vitamins, and/or iron. Your doctor also may suggest that you reduce the amount of fatty foods in your diet and increase your carbohydrate intake.
Weak or scarred muscles in the colon wall make it difficult for the bowel to work well, resulting in constipation or other abnormalities of the colon. Maintaining a diet high in fiber, and drinking at least six-to-eight glasses of fluids daily, especially water will help prevent constipation. Fresh fruits and vegetables are natural laxatives. Exercise also helps to keep bowel movements regular. Your doctor also may recommend stool softeners and bulking agents like Metamucil®.
Multiple factors can cause lung involvement in systemic scleroderma. Build-up of collagen thickens lung tissue and causes fibrosis or scarring, making the transport of oxygen into the bloodstream more difficult. Pulmonary arterial hypertension (PAH), a state of increased resistance to blood flow through the lungs, can result from damage to blood vessels, and may lead to additional strain on the heart resulting in heart failure. Respiratory muscle weakness may decrease lung function.
Symptoms of lung involvement include shortness of breath, a decreased tolerance for exercise and a persistent cough. The physician may order a chest X-ray, an echocardiogram (ultrasound of the heart), special breathing tests (pulmonary function tests) or a CAT scan of the lungs to detect or confirm lung involvement.
In the early stages of lung fibrosis, medications may be given to decrease the inflammation, which is thought to lead to lung scarring.
Although many investigations are under way, there currently are no proven medications to reverse lung changes once they have occurred. It is important, therefore, for the person with scleroderma to take whatever measures are within his or her control to avoid further damage to the lungs. It is essential to avoid smoking, a major cause of lung disease. Exposure to air pollutants may worsen breathing problems and should be avoided to the extent possible. Your doctor may recommend medications to make breathing easier and may suggest deep breathing exercises and a graduated aerobic exercise program.
People with pulmonary arterial hypertension may be treated with special medications targeted at dilating or opening up the blood vessels of the lungs, and possibly changing the underlying nature of the disease. This is one complication of scleroderma for which new medications have proven successful; there are now three different classes of medications, which can be used.
Raynaud Phenomenon is the most common early symptom of systemic scleroderma. It is present at one time or another in about 90 percent of patients. It is most obvious in the fingers and toes but can also involve the ears, nose and tip of the tongue. In Raynaud Phenomenon, the blood vessels constrict or narrow in response to cold or emotional upset and stress. The resulting disturbance in blood circulation causes a series of color changes in the skin: white, blanched or pale, when circulation is reduced; blue as the affected part loses oxygen from decreased blood flow; and then red or flushed as blood flow returns and the part re-warms. Finally, as the attack subsides and the circulation returns to normal, usual skin color is restored. In the “white” or “blue” stages, sensations such as tingling, numbness and coldness may be felt. In the “red” stage, a feeling of warmth, burning or throbbing may be noted. Some people find Raynaud attacks painful.
Many common-sense preventive measures can be taken by those susceptible to Raynaud Phenomenon. Most obvious is minimizing exposure to cold, such as outdoor weather, air conditioning, or reaching into a refrigerator or freezer. Keeping your extremities and body warm is very important. Gloves or mittens should be worn, and a number of warming devices are available to protect the hands. Hats, earmuffs, heavy socks and warm, layered clothing of made from silk, cotton, wool and down feathers can help maintain body temperature. It is important to protect your hands with gloves when touching refrigerated or frozen items. Electric heaters, electric blankets and comforters can supplement the heat in the home or apartment. Keeping the entire body warm helps prevent Raynaud episodes. A warm bath or shower, or heating pad or hot water bottle on the back, may relieve an attack better than just warming the hands. Avoidance of emotional upset and stress can help but isn’t always possible.
Raynaud Phenomenon is not confined to people with scleroderma. It is also seen in people with lupus, rheumatoid arthritis and other connective tissue diseases. In addition, many healthy people have Raynaud Phenomenon without any other illness. In this situation, it is called Primary Raynaud Disease.
Sjögren Syndrome (dry eyes or mouth) is characterized by a decrease in secretions of the tear glands and the salivary glands, which provide lubrication for the eyes and mouth. The unusual dryness of the eyes resulting from this condition can lead to serious irritation and inflammation. Excessive dryness of the mouth may lead to difficulties in swallowing and speaking, a pronounced increase in tooth decay and cavities, and a reduced sense of taste. The lack of secretions in Sjögren Syndrome also may involve the vagina and other areas of the body.