Research and continuing advances in treatment give people with scleroderma hope for a healthier future. Being better informed can help you be a more effective advocate in the management of your disease and remain as healthy as possible.
Raynaud’s phenomenon is a common medical condition and women are much more likely to develop Raynaud’s phenomenon than men. Most people with Raynaud’s will NOT develop scleroderma. On the other hand, nearly 90% of those with scleroderma have Raynaud’s symptoms. For this reason, it is a “red flag” that can help lead to earlier diagnosis and treatment.
Raynaud’s phenomenon is a condition that results in decreased blood flow to your fingers and toes resulting in color changes. In this condition, excess collagen in the vessel walls narrows the blood vessels.
During an attack, your body limits blood flow to the hands and feet. This makes your fingers and toes feel cold and numb. It may also cause your fingers to turn white or blue. Once blood flow to the fingers and toes returns, they may turn red, tingle, throb, or swell and begin to hurt. An attack usually lasts a few minutes. For some people, it may last more than an hour.
Persons with Raynaud’s phenomenon should avoid exposure to cold, stress, and stressful events. They should avoid medications that constrict blood vessels. These include some treatments for migraine, menopause, and attention-deficit disorder. Other substances that constrict blood vessels include caffeine, nicotine, amphetamines, and cocaine.
How to avoid a Raynaud’s attack:
- Keep your entire body warm, not just your hands and feet
- Always bring extra layers of clothing with you
- Stash extra mittens everywhere (in the kitchen for reaching into the refrigerator or freezer, on trips to the grocery store, etc.)
- Wear hats in cold weather
- Wear mittens (not gloves) and warm socks when the temperature drops below 65 degrees. Use chemical or battery-operated hand and foot warmers
- Wear comfortable shoes and socks that allow for good circulation
- Don’t hold cold drinks
- Use a space heater in the bathroom or at your office at work
- Warm up your bed with an electric blanket, hot water bottle or use flannel sheets
- Warm up your car before you leave the house; Use a remote car starter; Cover your steering wheel with a lambskin wheel cover
- Try to avoid sudden changes in temperature
- Avoid stressful situations
Treatments for Raynaud’s phenomenon
Consult with your doctor or rheumatologist regarding possible treatment with medications* which may include:
- Calcium channel blockers, which relax and open blood vessels
- Alpha blockers which work to prevent blood vessels from constricting
- Other vasodilators, which relax blood vessels to keep them open
- Nerve surgery to interrupt the signals that control the opening and narrowing of blood vessels
- Injections of local anesthetics to block the nerves in hands and feet
*Pregnant woman should not take these medications
For people with scleroderma, skin ulcers most often appear at the tips of fingers (digital ulcers), may extend under the nails, or to the joints at pressure points like elbows when the skin is stretched too tight. Ulcers may also occur on the legs or other parts of the body following minor trauma such as a bruise.
Skin ulcers are caused when excess collagen collects on the inside of blood vessels causing them to narrow and restrict blood flow. These sores may be very painful and can make it difficult to use your hands or other parts of your body.
Skin ulcers occur in about 50% of those with limited or diffuse scleroderma. Patients with repeated episodes of Raynaud’s phenomenon are also at risk for ulcers on the fingers or toes.
How Can I Prevent Skin Ulcers?
- Avoid stress and cold
- Keep as warm as possible
- Wear layers of clothing
- Wear hats in cold weather
- Wear shoes and socks that provide room for circulation
- Use Band-Aids
Treatments for Skin Ulcers:
Basic wound care and over-the-counter (OTC) medications may help with healing and pain management. Medications may also be recommended. These include:
- Calcium channel blockers
- Endothelin receptor blockers
More serious cases may require wound care, treatment of infections, and medication to improve blood flow. If the tissue is too damaged to heal, your doctor may recommend surgery or amputation might be needed.
Calcinosis is the abnormal collection of calcium salts which form under the skin and in muscles or tendons. This scleroderma complication can develop slowly over time and have no other symptoms, or can come on suddenly and be severe.
Calcinosis appears as hard, irregular nodules in or under the skin in any area of the body. These deposits vary in size and shape, ranging from a tiny speck, pea-sized, to larger tumorous deposits located most commonly in the fat pad of the fingertips, as well as on the hands, arms, elbows, knees, and trunk. They can also break the surface of the skin, becoming a lesion and can be skin-colored or white, hard or soft. Some might leak a white fluid and in severe cases can be very painful and limit range of motion.
Treatments for Calcinosis
Treatment of calcinosis from scleroderma can be challenging. There is no treatment that is effective for everyone and few medications or treatments are effective against calcinosis.
Increasing blood flow to the extremities, through smoking cessation, decreasing stress, and limiting exposure to cold, may be helpful. Appropriate sun-protection is always important since sun exposure can stimulate the immune system, which may contribute to calcinosis.
Lesions may respond to the following medications:
- Antacids can reduce risk by helping to prevent the absorption of phosphate
- Bisphosphonates help hinder bone turnover and lower calcium and phosphate levels
- Calcium channel-blockers can diminish the concentration of intracellular calcium
Surgical excision may be used to remove smaller lesions that are confined to a single area, especially those in painful or troubling areas of the body. Unfortunately, there is always a risk that the lesions will return. Calcinosis occasionally clears on its own with no intervention.
Telangiectasias are abnormal blood vessels that usually appear as red dots on the skin or may look like varicose veins, with a spider-web form. Patients with scleroderma may develop telangiectasias on the arms, hands, fingers, the face, chest, and in the mouth. Other locations might be over joints, and around the toenails or fingernails. They will fade or turn white when the skin is pressed and are not painful or dangerous.
The condition occurs with both limited and diffuse scleroderma. Telangiectasias in scleroderma become more numerous over time in both types of the disease, however, evidence suggests they occur more frequently in patients with limited scleroderma (CREST).
Treatments for Telangiectasia in Scleroderma
A pulsed dye laser can reduce the appearance of telangiectasias. It targets only the blood vessel and not the surrounding skin. A dermatologist might perform this treatment.
Scleroderma can cause scar tissue in the lungs and thickening in the walls of blood vessels that carry blood to and from the heart to the lungs. When the blood is less able to flow smoothly it can lead to pulmonary arterial hypertension (PAH) which is a life-threatening condition. PAH is a leading cause of disability and death among these patients with systemic sclerosis. It occurs in 8%-17% of patients and accounts for 30% of deaths because PAH symptoms are often not recognized early.
Part of the problem is that there are few very early symptoms of PAH. These are often mistaken for a general lack of fitness. Regular screening is very important, because people with scleroderma are at high risk for PAH.
Symptoms that develop are often typical of other conditions (such as asthma). This can cause a further delay in diagnosis. Patients may experience:
- Shortness of breath during normal activities
- Tiredness or fatigue
- Chest pain
- Rapid heartbeat
- Pain in the upper right side of the abdomen
- Decreased appetite
- Swelling of feet, ankles, legs
- Fainting, dizziness, light-headedness
- A bluish color in the lips and skin
Treatments for Pulmonary Arterial Hypertension (PAH)
Treatment for PAH is more effective if it is identified in earlier stages. Sadly, patients rarely report early symptoms such as shortness of breath and fatigue. They may be severely affected by the time their PAH is diagnosed.
Your doctor should be monitoring you regularly to check on the health of your heart and lungs. It is important to have regular screening for heart and lung function. Pulmonary function tests, echocardiography, chest X-rays, or CT scans can help identify problems. You may be referred to a pulmonary physician for evaluation and treatment recommendations.
There is no cure for PAH, but treatment can help to relieve symptoms, improve function, and slow the progression of the disease. Endothelial cells have many functions in our blood vessels. Endothelial cell dysfunction was discovered to be a key trigger for the development of vascular damage in systemic sclerosis. PAH is a major consequence of this damage. This discovery led to development of several treatment options over the past 20 years.
If you do develop PAH, your doctor can recommend a treatment that suits you. Medications for scleroderma-related PAH may be injected, inhaled, or taken by mouth. The goal of these treatments is to relax the blood vessels in the lungs and reduce excess cell growth, so that blood can flow more easily.
Interstitial Lung Disease (ILD) is another complication of systemic sclerosis which may affect breathing. ILD occurs when there is inflammation and scarring to the lung tissue that supports the alveoli—the air sacs within the lungs. Over time, this scarring, called pulmonary fibrosis, makes it difficult to breathe in and out, and reduces the amount of oxygen that can reach the bloodstream. About 15% of patients with systemic sclerosis have ILD.
Symptoms may include:
- Shortness of breath during normal activities
- Difficulty taking deep breaths
- Increased fatigue
- Dry cough that you can’t get rid of
- Feeling dizzy
- Widening of the fingertips
- Muscle and joint pain
There are several factors that are thought to trigger ILD development, or cause it to worsen:
- Inflamed lung tissue
- Scarred lung tissue
- Lung infections
- Occupational exposures to asbestos, coal or grain dust, mold, or silica dust
- Medications such as chemotherapy or immunomodulating drugs, heart medications, and some antibiotics
Treatments for Interstitial Lung Disease (ILD)
The scarring of lung tissue caused by ILD cannot be reversed. There are treatments to improve scleroderma Interstitial Lung Disease symptoms, slow the progression of the disease, and improve patients’ quality of life.
- Those with scleroderma need regular screening for pulmonary lung function. Patients may be referred to a pulmonologist for further assessment and treatment.
- Doctors will measure blood oxygen levels at rest and with activity. If they are low, supplementary oxygen may be prescribed.
- Lung transplantation may be suggested to patients with severe ILD with significant scarring.
Other symptoms may include:
- Stomach Pain
- Difficulty swallowing, and acid reflux
- Heartburn or indigestion.
- Changes in appetite.
- Fecal incontinence
Problems with the esophagus that result in swallowing difficulties and heartburn are the most common risks among people with scleroderma. Other risks can include:
- Gastroesophageal reflux disease or GERD. It is also called acid indigestion or heartburn. It occurs when stomach acids back up into the esophagus and cause irritation. With reflux, a person may inhale stomach acids into the lungs. This is irritating to the lungs and can complicate lung disease.
- Abnormal cell growth can damage the esophagus. This can cause swallowing difficulties. It can affect how well the esophagus moves food to the stomach. In severe cases may cause blockage or increase the risk of esophageal cancer.
- Damage to the intestines hinders the absorption of nutrients. It may cause substantial weight loss and malnutrition.
The GI system is complex. Treating GI symptoms is important but diagnosis and treatment will differ according to the location of the symptom or problem.
There are certain foods you may want to avoid in order to reduce acid reflux and heartburn, although experimentation may be needed to determine which food triggers symptoms.
- Caffeine, coffee (regular and decaf)
- Acidic foods (citrus fruits like oranges, tomato sauce)
- Fried foods
- Raw vegetables
- Foods with high fat content (fast foods, nuts, dairy products)
- Spicy foods
Many people with scleroderma are at risk for malnutrition. Regular assessment of nutritional status should be part of the treatment plan.
Development of fibrotic tissue can affect movement. Additionally, scarring and thickening of the skin can cause painful contractures of the fingers, wrists, or other joints, causing them to become “stuck” in a flexed position.
Aspirin or other nonsteroidal, anti-inflammatory prescriptions can help with muscle pain and stiffness. Simple hand and face exercises may ease specific symptoms and gentle cardiovascular exercise that focuses on your entire body (walking, swimming, yoga) can also help.
- Shortness of breath. This can be a sign of heart failure
- Night-time breathing difficulty
- Swollen feet and legs
- Liver enlargement
- Chest pain
In addition, some cardiac symptoms may not be recognized. They might be thought to be caused by pulmonary, musculoskeletal, or esophageal involvement.
In systemic scleroderma, the kidneys can be damaged due to a sudden and severe increase in blood pressure. This is called scleroderma renal crisis (SRC).
Scleroderma Renal Crisis is very serious. There are risks that can help identify it early. Risks include:
- Rapidly progressing diffuse systemic sclerosis, often within the first year.
- Having tendon friction rubs
- Prior use of high dose corticosteroids
- Presence of anti-RNA polymerase III
- New anemia, a possible early clue to the onset of SRC
- Cardiac events, like cardiac insufficiency or pericardial effusion, may precede SRC
Sudden onset of high blood pressure (hypertension) of more than 150/85mmHg is the key symptom of renal crisis. It causes an almost immediate decrease in kidney function.
Symptoms to watch for are:
- Visual disturbances
- General malaise
- Changes in blood or urine chemistry
- Pulmonary edema (fluid in the lungs)
One class of antihypertensive medications known as angiotensin converting enzyme inhibitors (ACE inhibitors) are effective, although many patients require more than one drug. If the blood pressure can be promptly and completely controlled, kidney injury can be prevented and over time can reverse.
What can you do?
- Learn how to measure your blood pressure and do so at least every other day
- Avoid or minimize the use of Corticosteroids and nonsteroidal anti-inflammatory drugs
- Use caution with cold exposure as it may reduce the risk
I’ve Been Diagnosed, What Do I Do?
There is a lot to understand about this complicated disease. Here is a place to get started.
Patients who are diagnosed with scleroderma are encouraged to receive care at a specialized Treatment Center.