Treatment

Currently, there is no treatment that controls or stops the underlying problem - the overproduction of collagen - in all forms of scleroderma. Thus, treatment and management focus on relieving symptoms and limiting damage.

Here is a listing of the potential problems that can occur in systemic scleroderma and the medical and nonmedical treatments for them. These problems do not occur as a result or complication of localized scleroderma. This listing is not complete because different people experience different problems with scleroderma and not all treatments work equally well for all people. Work with your doctor to find the best treatment for your specific symptoms.

Raynaud’s Phenomenon

More than 90 percent of people with scleroderma have this condition, in which the fingers and sometimes other extremities change color in response to cold temperature or anxiety. For many, Raynaud’s phenomenon precedes other manifestations of the disease. In other people, however, Raynaud’s phenomenon is unrelated to scleroderma, but may signal damage to the blood vessels supplying the hands arising from occupational injuries (from using jackhammers, for example), trauma, excessive smoking, circulatory problems, and drug use or exposure to toxic substances. For some people, cold fingers and toes are the extent of the problem and are little more than a nuisance. For others, the condition can worsen and lead to puffy fingers, finger ulcers, and other complications that require aggressive treatment.

If you have Raynaud’s phenomenon, the following measures may make you more comfortable and help prevent problems:

• Don’t smoke! Smoking narrows the blood vessels even more and makes Raynaud’s phenomenon worse.

• Dress warmly, with special attention to hands and feet. Dress in layers and try to stay indoors during cold weather.

• Use biofeedback, which governs various body processes that are not normally thought of as being under conscious control, and relaxation exercises.

• For severe cases, speak to your doctor about prescribing drugs called calcium channel blockers, such as nifedipine, which can open up small blood vessels and improve circulation. Other drugs are in development and may become available.

• If Raynaud’s leads to skin sores or ulcers, increasing your dose of calcium channel blockers (under the direction of your doctor ONLY) may help. You can also protect skin ulcers from further injury or infection by applying nitroglycerine paste or antibiotic cream. Severe ulcerations on the fingertips can be treated with bioengineered skin.

Stiff, painful joints

In diffuse systemic sclerosis, hand joints can stiffen due to hardened skin around the joints or inflammation within them. Other joints can also become stiff and swollen.

• Stretching exercises under the direction of a physical and/or occupational therapist are extremely important to prevent loss of joint motion. These should be started as soon as the diagnosis of scleroderma is made.

• Exercise regularly. Ask your doctor or physical therapist about an exercise plan that will help you increase and maintain range of motion in affected joints. Swimming can help maintain muscle strength, flexibility, and joint mobility.

• Use acetaminophen or an over-the-counter or prescription nonsteroidal anti-inflammatory drug, as recommended by your doctor, to help relieve joint or muscle pain. If pain is severe, speak to a rheumatologist about the possibility of prescription-strength drugs to ease pain and inflammation.

• Learn to do things in a new way. A physical or occupational therapist can help you learn to perform daily tasks, such as lifting and carrying objects or opening doors, in ways that will put less stress on tender joints.

Skin problems

When too much collagen builds up in the skin, it crowds out sweat and oil glands, causing the skin to become dry and stiff. If your skin is affected, you may need to see a dermatologist. To ease dry skin, try the following:

• Apply oil-based creams and lotions frequently, and always right after bathing.

• Apply sunscreen before you venture outdoors, to protect against further damage from the sun’s rays.

• Use humidifiers to moisten the air in your home in colder winter climates. Clean humidifiers often to stop bacteria from growing in the water.

• Avoid very hot baths and showers, as hot water dries the skin.

• Avoid harsh soaps, household cleaners, and caustic chemicals, if at all possible. Otherwise, be sure to wear rubber gloves when you use such products.

• Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.

Dry mouth and dental problems

Dental problems are common in people with scleroderma for a number of reasons. Tightening facial skin can make the mouth opening smaller and narrower, which makes it hard to care for teeth; dry mouth due to salivary gland damage speeds up tooth decay; and damage to connective tissues in the mouth can lead to loose teeth. You can avoid tooth and gum problems in several ways:

• Brush and floss your teeth regularly. If hand pain and stiffness make this difficult, consult your doctor or an occupational therapist about specially made toothbrush handles and devices to make flossing easier.

• Have regular dental checkups. Contact your dentist immediately if you experience mouth sores, mouth pain, or loose teeth.

• If decay is a problem, ask your dentist about fluoride rinses or prescription toothpastes that remineralize and harden tooth enamel.

• Consult a physical therapist about facial exercises to help keep your mouth and face more flexible.

• Keep your mouth moist by drinking plenty of water, sucking ice chips, using sugarless gum and hard candy, and avoiding mouthwashes with alcohol. If dry mouth still bothers you, ask your doctor about a saliva substitute, or prescription medications such as Salagen or Evoxac, that can stimulate the flow of saliva.

Gastrointestinal (GI) problems

Systemic sclerosis can affect any part of the digestive system. As a result, you may experience problems such as heartburn, difficulty swallowing, early satiety (the feeling of being full after you’ve barely started eating), or intestinal complaints such as diarrhea, constipation, and gas. In cases where the intestines are damaged, your body may have difficulty absorbing nutrients from food. Although GI problems are diverse, here are some things that might help at least some of the problems you have:

• Eat small, frequent meals.

• To keep stomach contents from backing up into the esophagus, stand or sit for at least an hour (preferably two or three) after eating. When it is time to sleep, keep the head of your bed raised using blocks.

• Avoid late-night meals, spicy or fatty foods, alcohol, and caffeine, which can aggravate GI distress.

• Eat moist, soft foods, and chew them well. If you have difficulty swallowing, or if your body doesn’t absorb nutrients properly, your doctor may prescribe a special diet.

• Ask your doctor about prescription medications for problems such as diarrhea, constipation, and heartburn. Some drugs called proton pump inhibitors are highly effective against heartburn. Oral antibiotics may stop bacterial overgrowth in the bowel that can be a cause of diarrhea in some people with systemic sclerosis.

Lung damage

Virtually all people with systemic sclerosis have some loss of lung function. Some develop severe lung disease, which comes in two forms: pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). Treatment for the two conditions is different:

• Pulmonary fibrosis may be treated with drugs that suppress the immune system such as cyclophosphamide (Cytoxan) or azathioprine (Imuran), along with low doses of corticosteroids.

• Pulmonary hypertension may be treated with drugs that dilate the blood vessels such as prostacyclin (Iloprost), or with newer medications that are prescribed specifically for treating pulmonary hypertension.

Regardless of your particular lung problem or its medical treatment, your role in the treatment process is essentially the same. To minimize lung complications, work closely with your medical team. Do the following:

• Watch for signs of lung disease, including fatigue, shortness of breath or difficulty breathing, and swollen feet. Report these symptoms to your doctor.

• Have your lungs closely checked, using standard lung-function tests, during the early stages of skin thickening. These tests, which can find problems at the earliest and most treatable stages, are needed because lung damage can occur even before you notice any symptoms.

• Get regular flu and pneumonia vaccines as recommended by your doctor. Contracting either illness could be dangerous for a person with lung disease.

Heart problems

Common among people with scleroderma, heart problems include scarring and weakening of the heart (cardiomyopathy), inflamed heart muscle (myocarditis), and abnormal heart beat (arrhythmia). All of these problems can be treated. Treatment ranges from drugs to surgery, and varies depending on the nature of the condition.

Kidney problems

Renal crisis occurs in about 10 percent of all patients with scleroderma, primarily those with early diffuse scleroderma. Renal crisis results in severe uncontrolled high blood pressure, which can quickly lead to kidney failure. It’s very important that you take measures to identify and treat the hypertension as soon as it occurs. These are things you can do:

• Check your blood pressure regularly. You should also check it if you have any new or different symptoms such as a headache or shortness of breath. If the blood pressure is higher than usual, call your doctor right away.

• If you have kidney problems, take your prescribed medications faithfully. In the past two decades, drugs known as ACE (angiotensin-converting enzyme) inhibitors, including captopril (Capoten), enalapril (Vasotec), or lisinopril, have made scleroderma-related kidney failure a less threatening problem than it used to be. But for these drugs to work, you must take them as soon as the hypertension is present.

Cosmetic problems

Even if scleroderma doesn’t cause any lasting physical disability, its effects on the skin’s appearance - particularly on the face - can take their toll on your self-esteem. Fortunately, there are procedures to correct some of the cosmetic problems scleroderma causes:

• The appearance of telangiectasias - small red spots on the hands and face caused by swelling of tiny blood vessels beneath the skin - may be reduced or even eliminated with the use of guided lasers.

• Facial changes of localized scleroderma - such as the en coup de sabre that may run down the forehead in people with linear scleroderma - may be corrected through cosmetic surgery. (However, such surgery is not appropriate for areas of the skin where the disease is active.)

Reference:

National Institute of Arthritis and Musculoskeletal and Skin Diseases, USA.