Education
Treatment of Scleroderma

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 focuses on relieving symptoms and limiting damage. Your treatment will depend on the particular problems you are having. Some treatments will be prescribed or given by your doctor. Others are things you can do on your own.  Work with your health care professionals, experienced in the care of people living with scleroderma, in order to develop a personalized treatment plan.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors involved in your care. Typically, care will be managed by a rheumatologist (a doctor specializing in treatment of musculoskeletal disorders and rheumatic diseases). Your rheumatologist may refer you to other specialists, depending on the specific problems you are having. For example, you may see a nephrologist for kidney complications, a cardiologist for heart complications, a gastroenterologist for problems of the digestive tract, and a pulmonary specialist for lung involvement.

In addition to doctors, professionals such as nurse practitioners, physical or occupational therapists, psychologists, patient educators, and social workers may play a role in your care. Dentists, orthodontists, and even speech therapists can treat oral complications that arise from thickening of tissues in and around the mouth and on the face.

Below are descriptions of potential implications of systemic sclerosis.

Raynaud’s Phenomenon

More than 90 percent of persons with scleroderma experience Raynaud’s Phenomenon in which the fingers and sometimes other extremities change color in response to cold temperature or anxiety. In most cases, Raynaud’s precedes other manifestations of the scleroderma. However, Raynaud’s Phenomenon itself may be 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, drug use, or exposure to toxic substances. For some, 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 including amputation.

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 worse.
  • Dress warmly and pay particular attention to maintaining your core body temperature.  Wearing gloves when fingers are in direct contact with cold (for example, when holding a non-insulated glass with ice cubes or getting something cold out of the freezer) is important.
  • Use biofeedback and relaxation exercises.
  • For severe cases, consult with your doctor. There are prescription medications available that are designed to open up small blood vessels and improve circulation. Should Raynaud’s Phenomenon lead to skin sores or ulcers, protecting skin ulcers from further injury or infection is critically important. Severe ulcerations on the fingertips may be treated with bioengineered skin or surgical interventions.

For more information, download the Scleroderma Patient Education Booklet "Raynaud's, Finger and Skin Care" developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Skin

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, 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.

For more information, download the Scleroderma Patient Education Booklet "Raynaud's, Finger and Skin Care" developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Joints, Pain & Fatigue

In diffuse systemic sclerosis, hand joints can stiffen because of 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 or occupational therapist are extremely important to prevent loss of joint motion. These should be started as soon as scleroderma is diagnosed.
  • 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.
  • 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.

For more information, download the Scleroderma Patient Education Booklet “Pain and Fatigue” developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Oral, Dental & Facial Health

Tightening facial skin may make the mouth opening smaller and narrower making it difficult to properly care for teeth. Dry mouth caused by salivary gland damage speeds up tooth decay and damage to connective tissues in the mouth can lead to loose teeth. You may 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 re-mineralize 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 remains an issue, ask your doctor about a saliva substitute—or prescription medications such as pilocarpine hydrochloride or cevimeline hydrochloride—that can stimulate the flow of saliva.
  • Excessive dryness of the mouth may be associated with the presence of Sjogren'sSyndrome leading to increased dental caries (cavities), difficulty eating, and difficulty speaking. With Sjogren'sSyndrome, patients may experience a reduction in tears. Keep your eyes lubricated with saline eye drops as recommended by your physician.

For more information, download the Scleroderma Patient Education Brochure “Oral and Dental Problems” developed by the Scleroderma Australia by clicking here

Gastrointestinal (GI) System

Systemic sclerosis may affect the digestive system. You may experience 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. Where the intestines are damaged, your body may have difficulty absorbing nutrients from food.

  • Eat small, frequent meals.
  • To prevent stomach contents from backing up into the esophagus, stand or sit for at least an hour (preferably 2 or 3 hours) after eating.
  • For sleep, keep the head of your bed raised by slipping a pillow or brick between the box spring and mattress.
  • Avoid late-night meals, spicy or fatty foods, alcohol, and caffeine.
  • 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 diarrhea, constipation, and heartburn. Proton pump inhibitors (PPIs) are highly effective for heartburn. Oral antibiotics may stop bacterial overgrowth in the bowel, which can be a cause of diarrhea in some people with systemic sclerosis.

For more information, download the Scleroderma Patient Education Booklet “Gastrointestinal Tract” developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Lungs

Virtually all persons with systemic sclerosis have some loss of lung function. Lung disease comes in two forms:

  • Pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) which may be treated with drugs that suppress the immune system, such as cyclophosphamide or azathioprine, along with low doses of corticosteroids; or
  • Pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs) which may be treated with drugs that dilate the blood vessels, such as prostacyclin, or with newer medications that are prescribed specifically for treating pulmonary hypertension.

Regardless of the type of lung involvement, work closely with your medical team.

  • 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 monitored using standard lung-function tests, during the early stages of skin thickening. These tests, which can identify 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.

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys” developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Kidneys

Renal crisis occurs in about 10 percent of patients with scleroderma and primarily those with early diffuse scleroderma. Severe uncontrolled high blood pressure can quickly lead to kidney failure. It is very important that you take measures to identify and treat hypertension as soon as it occurs.

  • Check your blood pressure regularly and if you develop new or different symptoms such as headaches or shortness of breath. If your blood pressure is higher than usual, call your doctor immediately.
  • Take your medications as prescribed. New medications known as ACE (angiotensin-converting enzyme) inhibitors, including captopril, enalapril, and lisinopril, have made scleroderma-related kidney failure a less threatening problem.

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys” developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Heart

Heart involvement include scarring and weakening of the heart (cardiomyopathy), inflamed heart muscle (myocarditis), and abnormal heartbeat (arrhythmia) are common in persons with scleroderma. Treatment may range from drugs to surgery.

For more information, download the Scleroderma Patient Education Booklet “Heart, Lungs & Kidneys” developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley by clicking here

Cosmesis

There are procedures available to improve the cosmetic issues commonly associated with scleroderma. 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 provided the disease is not active.

Living with Scleroderma

Scleroderma may affect aspects of daily living from family relationships to holding a job to the ability to dress, bathe, or handle the most basic daily tasks.

Appearance and Self-esteem
People with scleroderma commonly express concern with how the disease will affect their appearance. Thick, hardened skin may be difficult to accept, particularly on the face. Systemic scleroderma may result in facial changes that eventually cause the opening to the mouth to become smaller and the upper lip to virtually disappear. Linear scleroderma may leave its mark on the forehead. Although these problems can’t always be prevented, their effects may be minimized with proper treatment. Special cosmetics - and in some cases plastic surgery - may help conceal skin damage.

Caring for Yourself
Tight, hard connective tissue on the hands may make it difficult to do what were once simple tasks, such as brushing your teeth and hair, pouring a cup of coffee, using a knife and fork, unlocking a door, or buttoning a jacket. If you have trouble using your hands, consult an occupational therapist, who may recommend new ways of doing things or devices to make tasks easier. Velcro fasteners and built-up brush handles may help with independence.

Family Relationships
Spouses, children, parents, and siblings may have trouble understanding why you don’t have the energy to keep house, drive to soccer practice, prepare meals, or hold a job. Encourage them to learn about scleroderma and to share information with family and friends. Attending a counseling session and/or a support group may help them better understand the disease and how they may help you.

Sexual Relations
Sexual relationships may be affected when systemic scleroderma enters the picture. For men, the disease’s effects on the blood vessels can lead to problems achieving an erection. For women, damage to the moisture-producing glands can cause vaginal dryness that may make intercourse painful. Either sex may have difficulty moving the way they once did. They may also be self-conscious about their appearance or afraid that their sexual partner will no longer find them attractive. With good communication, good medical care, and perhaps counseling, many of these changes can be overcome.

Pregnancy and Childbearing
In the past, women with systemic scleroderma were often advised not to have children. Thanks to better medical treatments and a better understanding of the disease itself, that advice is changing. (Pregnancy, for example, is not likely to be a problem for women with localized scleroderma.) Although blood vessel involvement in the placenta may cause babies of women with systemic scleroderma to be born early, many women with the disease can have safe pregnancies and healthy babies if they take precautions that may include waiting a few years after the disease starts before attempting a pregnancy. During the first 3 years, you are at the highest risk of developing severe problems of the heart, lungs, or kidneys that may be harmful to you and your unborn baby. If you haven’t developed severe organ problems within 3 years of the disease’s onset, your chances of such problems are less and pregnancy would be safer. It is important to have both your disease and your pregnancy monitored regularly. Stay in close contact with both the doctor you typically see for your scleroderma and an obstetrician who is experienced in managing high-risk pregnancies.

For more information about living with scleroderma, download the following Scleroderma Patient Education Booklets developed by behavioral psychologist Dr. Jennifer Haythornthwaite and Johns Hopkins Scleroderma Center director Dr. Fredrick Wigley and Drs. Maureen Mayes and Khanh Ho from the Scleroderma Foundation:

  • "Living with Scleroderma” by clicking here
  • "Psychological Impact of Scleroderma" by clicking here
  • "Understanding and Managing Scleroderma" booklet by clicking here

Treatment
At this time there is no cure for scleroderma. In most cases, localized scleroderma carries a good prognosis and patients have a normal life span. Most patients with limited CREST scleroderma can also expect a favourable outlook. Although the course of this disease tends to be slowly progressive, in time many internal organs may become affected.

With diffuse scleroderma, the severity varies widely so it is difficult to predict its course. It generally follows one of two paths:

  1. it is either acute and rapidly progresses into a life threatening condition; or

  2. it follows a very slow course.

The most critical period for systemic sclerosis is within the first years of onset. This is when there is the highest risk for rapid progression and the development of serious complications, particularly if the lungs, heart, or kidneys are affected.
Complete spontaneous improvement is very rare. Symptoms can be improved with medication and life style changes. Some medications are aimed at specific symptoms, while others are aimed at decreasing the activity of the immune system. Because of the advances in treatment, patient survival has improved a great deal over the past years.

References

  • The National Institute on Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, U.S. Department of Human and Health Services.
  • The Scleroderma Foundation in consultation with Dr. Maureen Mayes, M.D.,M.P.H., University of Texas/Houston and Dr. John Varga, M.D., Northwestern University.
Stay In Touch With Us

Make A Difference

Leave A Legacy

Support our mission with a charitable donation and enjoy the tax benefits. We have a number of giving opportunities for you to choose from.