Scleroderma / Systemic Sclerosis
A chronic connective tissue disease that’s generally classified as one of the autoimmune rheumatic diseases.Hardening skin is one of the most visible manifestations of the disease. The disease was called “progressive systemic sclerosis,” but term is discouraged since scleroderma is not necessarily progressive. This disease varies from patient-to-patient. Scleroderma is not contagious, infectious, cancerous or malignant disease.
How serious is Scleroderma / Systemic Sclerosis?
As with any chronic disease, it can be serious. The symptoms vary greatly for each person and the effects of scleroderma can range from very mild to life threatening. Depending on the parts of the body that are affected and the extent to which they are affected, the seriousness will vary. A mild case can become more serious if it’s not properly treated. Quick and proper diagnosis and treatment by qualified physicians can potentially minimize the symptoms of scleroderma and lessen the chances for irreversible damage.
What causes Scleroderma / Systemic Sclerosis?
It is still unknown what the exact cause or causes of scleroderma are but scientists and medical researchers are working hard to make those determinations. It is however, known that scleroderma involves an overproduction of collagen.
Is Scleroderma / Systemic Sclerosis genetic?
Most patients don’t have any family with scleroderma and is not passed to their children. Research indicates that there is a susceptibility gene. This raises the likelihood of developing scleroderma but by itself does not cause the disease.
What are the different types of Scleroderma / Systemic Sclerosis?
There are 2 major classifications of Scleroderma / Systemic Sclerosis:
- Localized scleroderma
- Systemic sclerosis (SSc)
Other forms or subclassifications, each with its own characteristics and prognosis may be identified through future research.
- Localized Scleroderma: The changes are usually found in only a few places on the skin or muscles and rarely spread elsewhere. It is normally relatively mild and internal organs are usually not affected and patients rarely develop systemic scleroderma. Some laboratory abnormalities commonly seen are frequently absent in the localized form.
- Morphea: A type of localized scleroderma that’s characterized by waxy patches on the skin of varying sizes, shapes and color. Skin under the patches may thicken. The patches itself may enlarge or shrink and may often disappear spontaneously. It usually appears between the ages of 20 and 50 but is often seen in young children.
- Linear scleroderma: A type of localized scleroderma that frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. It sometimes form a long crease on the head or neck (en coup de sabre) due to its resemblance to a saber or sword wound. It tends to involve deeper layers of the skin as well as the surface layers and sometimes affects the motion of the joints that lie underneath. It usually develops in childhood. The growth of involved limbs may be affected in children.
- Systemic scleroderma (systemic sclerosis): The changes may affect the connective tissue in many parts of the body. It can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. Blood vessels, muscles and joints may also be affected. The tissues of affected organs become hard and fibrous. This causes them to function less efficiently. There are 2 major recognized patterns that the illness can take; diffuse or limited disease. In diffuse scleroderma, thickening of the skin occurs faster and involves more skin areas than in limited disease. Additionally, people with diffuse scleroderma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs.
SYMPTOMS OF SCLERODERMA / SYSTEMIC SCLEROSIS
The signs and symptoms vary depending on which parts of your body are involved:
- Skin: Almost everyone who has scleroderma has experienced a hardening and tightening of patches of skin. The patches may be shaped like ovals or straight lines or they could cover wide areas of the trunk and limbs. The number, location and size of the patches vary by the type of scleroderma. Because it’s so tight and movement of the affected area may be restricted, the skin may appear shiny.
- Fingers or toes: An exaggerated response to cold temperatures or emotional distress which can cause numbness, pain or color changes in the fingers or toes is one of the earliest signs. Known as Raynaud’s disease, this condition can also occur in people who don’t have scleroderma.
- Digestive system: In addition to acid reflux, some people with scleroderma may also experience problems absorbing nutrients if their intestinal muscles aren’t moving food properly through the intestines.
- Heart, lungs or kidneys: This condition can affect the function of the heart, lungs or kidneys to varying degrees. If these problems are left untreated, they can become life threatening.
TREATMENT OPTIONS AVAILABLE FOR SCLERODERMA / SYSTEMIC SCLEROSIS
Skin problems tend to fade away on their own in 3 to 5 years in some cases. Scleroderma that affects internal organs normally worsen with time.
Currently, no drug has been developed that can stop the underlying process of scleroderma. However, there are a variety of medications that can help control scleroderma symptoms or help prevent complications. To accomplish this, these drugs may:
- Dilate blood vessels: Blood pressure medications may help to prevent lung and kidney problems and also to treat Raynaud’s disease.
- Suppress the immune system: Drugs that suppress the immune system such as those taken after organ transplant may also help reduce scleroderma symptoms.
- Reduce stomach acid: Medications like omeprazole (Prilosec) may relieve symptoms of acid reflux.
- Prevent infections: Antibiotic ointment, cleaning and protection for colds may help prevent infection of fingertip ulcers caused by Raynaud’s disease. Regular influenza and pneumonia vaccinations may help protect lungs that have been damaged by scleroderma.
- Relieve pain: If over-the-counter pain relievers isn’t effective enough, you can ask your doctor to prescribe stronger medications.
Physical or occupational therapists can help you to:
- Manage pain
- Improve your strength and mobility
- Maintain independence with daily tasks
As a last resort, surgical options for scleroderma complications include:
- Amputation: Amputation may be necessary if finger ulcers caused by severe Raynaud’s disease develop gangrene.
- Lung transplants: Patients who have developed high blood pressure in the arteries to their lungs (pulmonary hypertension) may be candidates for lung transplants.
DIAGNOSING SCLERODERMA / SYSTEMIC SCLEROSIS
Scleroderma can be difficult to diagnose as it can take so many forms and affect so many different areas of the body.
Your doctor may suggest blood tests to check for elevated blood levels of certain antibodies produced by the immune system after a thorough physical exam. A small tissue sample of the affected skin may be taken so that it can be examined for abnormalities.
Breathing tests (pulmonary function tests), a CT scan of your lungs and an echocardiogram of your heart may also be ordered.