Scleroderma is a connective-tissue disease that causes a progressive build up of tough scar-like tissue in the skin and internal organs. The term scleroderma is derived from the Greek words, skleros, meaning "hard," and derma meaning "skin." An individual with scleroderma may develop either a localized or systemic form of the disease. Localized scleroderma usually affects only the skin on the hands and face. Systemic scleroderma, however, affects the connective tissue in many parts of the body, including the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart, and other internal organs. It is unusual for localized scleroderma to progress to the systemic form. According to the Scleroderma Foundation, an estimated 300,000 people in the United States have the condition.
Signs and SymptomsSymptoms of scleroderma may include one or more of the following:
- Raynaud's phenomenon (abnormal sensitivity to cold in the hands or feet)
- Swelling of the fingers, hands, forearms, feet, lower legs, and face
- Thickening, hardening, and discoloration of the skin
- Ulcers or lesions on fingers, face, tongue, and inner lining of the cheek
- Joint pain, swelling, and stiffness (especially in the fingers and knees)
- Carpal tunnel syndrome
- Shortness of breath, cough
- Dry eyes
- Sexual dysfunction
- Digestive and gastrointestinal problems including difficulty swallowing, bloating, and abdominal pain
CausesScleroderma results from an overproduction of collagen, the primary connective tissue protein in the body. Scientists believe that the immune system mistakenly attacks the patient's own cells (an autoimmune response), producing too many white blood cells and other factors that cause a damaging inflammatory response and an overproduction of collagen, primarily in the skin. Researchers are not clear why this autoimmune response occurs, but they suggest that both genetic and environmental factors play a role in the development of the disease. For example, scleroderma has been associated with a number of industrial and pharmaceutical chemicals including:
- Silica dust
- Some plastic materials, such as epoxy resins and vinyl chloride
- Aromatic hydrocarbons
- L-tryptophan (synthetic supplement)
- Rapeseed oil
Risk FactorsThe following factors may increase a person's risk for scleroderma:
- Gender – approximately 3 times more women than men develop the disease
- Age – scleroderma is most common among individuals between the ages of 20 and 50; it is relatively rare in children
- Race/Ethnicity – young women of African ancestry and native Americans of the Choctaw tribe of Oklahoma have a high prevalence of the disease
DiagnosisAn individual with symptoms of scleroderma will most likely require consultations with both a rheumatologist (arthritis specialist) and a dermatologist (skin specialist). The physician will conduct a physical examination in which he or she may feel the skin, checking for thickened and hardened areas and may also press affected tendons and joints. The physician may also conduct the following procedures to diagnose the disease:
- Blood tests – may detect elevated levels of antibodies found in the majority of individuals with scleroderma
- Skin biopsy – samples of skin may be taken to identify abnormalities suggestive of scleroderma
- Chest X ray and/or pulmonary function test – may detect lung damage or altered lung capacity
- MRI and/or CT scan – often detect early signs of damage to the muscles and internal organs
Preventive CareIt is important for an individual with scleroderma to avoid developing infections, so the physician will administer the following:
- Pneumococcal pneumonia vaccine
- Annual flu vaccine
Treatment ApproachCurrently, there is no cure for scleroderma. While medications are often used to treat the symptoms of the disease, they are not always effective and many have significant side effects. There are, however, less toxic ways to treat the symptoms of scleroderma, which make living with the disease easier. For example, biofeedback successfully controls the temperature of the hands and feet of those with Raynaud's phenomenon. Studies also suggest that acupuncture may raise the temperature and improve circulation in the hands and feet. Finally, simple lifestyle changes may also improve an individual's quality of life.
LifestyleWhile lifestyle adjustments will not stop the progression of scleroderma, these simple measures may enhance an individual's quality of life:
- Eating small, frequent meals – may reduce gastrointestinal problems
- Maintaining an active lifestyle – may preserve muscle
- Avoiding smoke, exposure to cold, and stress – may prevent blood vessel spasms that diminish circulation
- Applying soothing skin creams to affected areas – may reduce pain, swelling, and stiffness
MedicationsLocalized scleroderma often is treated with topical therapies such as moisturizers or topical corticosteroids. Oral medications may also be used to halt the progression of localized scleroderma if it involves a large area of the body, such as an entire arm or leg. Systemic scleroderma may be treated with medications that improve circulation, promote gastrointestinal function, preserve kidney function, and control high blood pressure. Some medications a physician may prescribe for scleroderma include:
- Penicillamine – can reduce skin thickening and delay spread of damage to internal organs; has relatively high incidence of adverse reactions.
- Pentoxifylline – may improve circulation
- Immunosuppressant medications (such as cyclophosphamide) – often used for patients with life-threatening form of the disease; potent medications that damage cells' genetic information; may cause significant side effects
- Anti-inflammatory medications (such as aspirin, ibuprofen, and corticosteroids) – may reduce inflammation and swelling
- Calcium-channel blockers (such as nifedipine and diltiazem) – often used to treat Raynaud's phenomenon
- Antibiotics – prevent overgrowth of bacteria in the intestines and improve digestive processes
- ACE inhibitors (such as captopril) – may improve hypertension, prevent kidney damage, and dilate blood vessels
Surgery and Other ProceduresWhen symptoms of scleroderma become very severe, physicians may recommend the following procedures:
- Surgery to repair damage to the stomach or intestinal walls
- Amputation of severely diseased and infected fingers or toes
- Kidney, heart, and/or lung transplantation (in rare cases)
Nutrition and Dietary SupplementsMany individuals with scleroderma develop gastrointestinal problems and consequently reduce the amount of food they consume and nutrients they absorb. A recent study found that people with scleroderma tend to have deficiences in many vitamins and minerals. To prevent malnutrition, clinicians may recommend daily multivitamins that contain the following:
- Vitamin E
- Vitamin C
- Linoleic acid
HerbsAlthough herbs have not been rigorously tested for the treatment of scleroderma, some preliminary studies of particular herbs have shown promise in inhibiting actions that may be related to the development of scleroderma. These herbs include:
Danshen root (Salvia miltiorrhizae)
This herb is currently used in China to treat circulatory problems and kidney failure. One preliminary study indicates that danshen root prevents the formation of fibrous tissue, but further research is required before it can be proven effective for the treatment of scleroderma.
Laboratory studies indicate that this Chinese herbal mixture may inhibit collagen synthesis. The active ingredients in this herbal mixture include Ceylon cinnamon (Cinnamomum verum), Chinese cinnamon bark (Cinnamomum aromaticum) used traditionally for bloating and heartburn, and peony root (Paeonia lactiflora) used for arthritis as well as gastrointestinal, heart, and circulatory problems.
Gotu Kola (Centella asiatica)
Clinical trials suggest that low doses of gotu kola may decrease hardening of the skin associated with scleroderma, reduce joint pain and improve finger mobility. These studies involved small numbers of patients, however, so additional research in this area is warranted.
Although they haven't been scientifically examined for the treatment of scleroderma specifically, certain herbs, including bromelain (Ananas comosus), hawthorn berry (Crataegus monogyna), and horse chestnut (Aesculus hippocastanum), may be beneficial in treating connective tissue disorders in general.
AcupunctureA few studies of patients with systemic scleroderma indicate that acupuncture may improve circulation in the hands and fingers, mend fingertip ulcers, and possibly reduce the formation of fibrous tissue.
Massage and Physical TherapyResearch suggests that massage may be useful in improving circulation and preventing muscle distortion. More research is needed in this area to determine whether massage is truly an effective complementary therapy for scleroderma.
Mind/Body MedicineBiofeedback appears to successfully control the temperature in the hands and feet of those with Raynaud's phenomenon, a symptom often found in those with scleroderma.
Prognosis and ComplicationsPossible complications that may result from scleroderma include the following:
- Joint damage
- Damage to smooth muscles in the gastrointestinal tract
- Formation of fibrous tissue in the heart muscle; may lead to permanent damage and/or degradation
- Kidney damage and/or failure
- Formation of fibrous tissue in the thyroid gland
- The CREST syndrome tends to progress slowly and remains relatively benign for decades. The 10-year survival rate for those with CREST is 75%.
- Those with localized scleroderma also have a 10-year survival rate of 75%.
- Systemic scleroderma tends to progress faster in men and in those who are older at the onset of the disease. The 10-year survival rate for those with systemic scleroderma is 55%. If damage spreads to the heart, lung, or kidneys early in the course of the disease, prognosis is generally poor.
- Spontaneous remissions from scleroderma have also been reported, with symptoms of the disease resolving in the reverse order that they appeared.
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