Scleroderma is a chronic but rare autoimmune disease in which normal tissue is replaced with thick tissue with extra collagen. Although it most often affects the skin, scleroderma also can affect many other parts of the body.
Scleroderma is a chronic, although rare, autoimmune disease in which normal tissue is replaced with dense, thick fibrous tissue. Normally, the immune system helps defend the body against disease and infection. In patients with scleroderma, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in the skin and organs, which causes hardening and thickening (similar to the scarring process).
Although it most often affects the skin, scleroderma also can affect many other parts of the body including the gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles and joints. Scleroderma in its most severe forms can be life-threatening.
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There are 2 major forms of scleroderma, localized and systemic. Systemic scleroderma can be broken down into two main types: diffuse and limited.
The more common form of the disease, localized scleroderma, affects only a person's skin, usually in just a few places. It often appears in the form of waxy patches or streaks on the skin, and it is not uncommon for this less severe form to go away or stop progressing without treatment.
As its name implies, this form affects many parts of the body. Not only can it affect the skin, but it also can affect many internal organs, hindering digestive and respiratory functions, and causing kidney failure. Systemic scleroderma can sometimes become serious and life-threatening.
Also known as CREST syndrome, each letter stands for a feature of the disease:
Patients with limited scleroderma do not experience kidney problems. The skin thickening is restricted to the fingers, hands and forearms, and also sometimes the feet and legs. Digestive involvement is confined mostly to the esophagus. Among later complications, pulmonary hypertension, which can develop in 20% to 30% of cases, can be potentially serious. In pulmonary hypertension, the arteries from the heart to the lungs narrow down and generate high pressure on the right side of the heart, which can ultimately lead to right sided heart failure. Early symptoms of pulmonary hypertension include shortness of breath, chest pain, and fatigue.
Approximately 250 persons per million American adults are affected by scleroderma. It usually develops between the ages of 35 and 55, although a pediatric form also occurs. Scleroderma is four times more common in women than it is in men.
The exact cause of scleroderma is unknown. Although rarely, scleroderma can run in families. Most cases do not show any family history of the disease. Scleroderma is not contagious.
In addition to the thickening of skin, the following other symptoms may occur in a person with scleroderma:
In addition to these symptoms, 2 other conditions—Raynaud's phenomenon and Sjögren's syndrome—also affect patients with scleroderma. Approximately 85% to 95% of scleroderma patients experience Raynaud's phenomenon. However, primary Raynaud's phenomenon is common and often occurs by itself without any underlying connective tissue disorder. Only 10% of patients with Raynaud's phenomenon will develop scleroderma.
Sjögren's syndrome is manifested by dry eyes and mouth. This dryness is due to lack of secretion of tears and saliva as a result of immune damage and destruction of the moisture-producing glands of the body. This condition is named after the Swedish eye doctor, Henrik Sjögren, who first described it. It is seen in approximately 20% of patients with scleroderma.
After discussing your personal family medical history, your doctor will perform a thorough physical examination. In doing so, he or she will be looking for any of the symptoms mentioned above, especially thickening or hardening of the skin around the fingers and toes or discoloration of the skin. If scleroderma is suspected, tests will be ordered to confirm the diagnosis, as well as to determine the severity of the disease. These tests may include:
Currently, there is no cure for scleroderma. Instead, treatment is directed at controlling and managing the symptoms. Because scleroderma can have many symptoms, a combination of approaches is often needed to treat and manage the disease effectively.
For pulmonary hypertension, the most successful treatment is continuous intravenous infusion of epoprostenol (Flolan®), a prostaglandin, through a pump. Subcutaneous infusion of treprostinil (Remodulin®) – a related prostaglandin – is an acceptable alternative. Prostaglandins are hormone-like substances found in the body that, among other things, help relax the smooth muscle, and hence dilate blood vessels. Other forms of therapy that are currently FDA approved for pulmonary hypertension include oral bosentan (Tracleer ®), sildenafil (Revatio ®) and inhaled iloprost (Ventavis®).
Lung transplant is a viable option for both severe (drug refractory) interstitial lung disease and pulmonary hypertension.
In addition to taking prescribed medications correctly and regularly, there are many steps a person with scleroderma can take to better manage the disease. These include:
Regular exercise will not only help improve your overall physical and spiritual well-being, but it will also help keep your joints flexible and improve circulation. Consult your doctor or physical therapist for appropriate exercises.
When your joints hurt, avoid lifting heavy objects or performing chores that may place a strain on them, thus risking further injury. A physical therapist can help you learn new ways to perform daily activities without placing undue strain on your joints.
Taking proper precautions and care of your skin can be beneficial not only for symptoms of Raynaud's phenomenon, but also in taking care of the dry, thick patches of skin that result from localized scleroderma. There are many ways to accomplish this, including:
Aside from eating healthy foods to get the proper amounts of vitamins and nutrients, it is important to eat foods that do not aggravate existing stomach problems. Ways to do this include:
For those patients with scleroderma who also have Sjögren's syndrome, proper dental care is essential. Sjögren's syndrome increases the risk of developing cavities and tooth decay.
Because the effects of stress can play a part in reducing your blood flow, as well as affect many other aspects of your emotions and health, it is important to learn to manage or reduce stress. This can be done by taking the following steps:
Although no cure has been found for scleroderma, the disease is very often slowly progressive and manageable, and people who have it may lead healthy and productive lives. Like many other conditions, education about scleroderma and local support groups can be the greatest tools for managing the disease and reducing the risk of further complications.
Last reviewed by a Cleveland Clinic medical professional on 09/16/2019.
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