Scleroderma

Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body.

Left Arm Scleroderma

Risk factors

Anyone can get scleroderma; however, some groups have a higher risk of developing the disease. The following factors may affect your risk.

Abdomen affected by circumscribed scleroderma

• Sex. Scleroderma is more common in women than in men.
• Age. The disease usually appears between the ages of 30 and 50 and is more common in adults than children.
• Race. Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely. For example:
• The disease is more common in African Americans than European Americans.
• African Americans with scleroderma develop the disease earlier when compared with other groups.
• African Americans are more likely to have more skin involvement and lung disease when compared with other groups.

Lung Scleroderma

Types of Scleroderma

Localized scleroderma affects the skin and underlying tissues and generally appears in one or both of these patterns: Morphea, or patches of scleroderma that may be a half-inch or larger in diameter. Linear scleroderma, when the scleroderma thickening occurs in a line. This usually extends down an arm or leg, but sometimes runs down the forehead and face. Systemic scleroderma, sometimes called systemic sclerosis, affects your skin, tissues, blood vessels, and major organs. Doctors usually divide systemic scleroderma into two types: Limited cutaneous scleroderma, which comes on gradually and affects the skin on your fingers, hands, face, lower arms, and legs below the knees. Diffuse cutaneous scleroderma, which comes on more rapidly and starts as being limited to the fingers and toes, but then extends beyond the elbows and knees to the upper arms, trunk, and thighs. This type usually has more internal organ damage.

Symptoms of Scleroderma

• The symptoms of scleroderma vary from person to person depending on the type of scleroderma you have.
• The symptoms of scleroderma are different for each person, depending on the type of scleroderma you have.
• Localized scleroderma typically causes patches of thick, hard skin in one of two patterns:
• Patches in firm, oval shapes that stay in one area or spread to other areas of skin.
• Lines of thickened or different colored skin that run down your arm, leg, and, rarely, on the forehead.
• Systemic scleroderma, also known as systemic sclerosis, may come on quickly or slowly and may also cause problems with your internal organs in addition to the skin. Many people with this type of scleroderma feel overly tired (fatigue).

Linear morphea

Causes of Scleroderma

Several factors may contribute to the disease:

• Genetic makeup.
• Environment.
• Hormones.

Scleroderma - Interstitial fibrosis

Diagnosis of Scleroderma

Most children and teens with mild scoliosis do not have symptoms or pain. Sometimes, the child, teen, or a family member may notice changes in posture, which may be a sign of scoliosis. Other signs may include the following.

• Top of shoulders are uneven.
• One shoulder blade (in the upper back) is more prominent or visible than the other.
• One hip appears higher than the other.
• One side of the rib cage is higher than the other when bending forward.

If the scoliosis is progressing and the curve is more severe, the changes in the shape of the spine can lead to back pain. If the curve is severe, rarely, some children may develop difficulty breathing.

Treatment of Scleroderma

• Medications to help decrease swelling, manage pain, control other symptoms, and prevent complications that may arise.
• Physical or occupational therapy to help with pain, improve muscle strength, and teach ways to help with daily living, such as brushing your teeth.
• Regular dental care because scleroderma can make your mouth dry and damage connective tissues in your mouth, speeding up tooth decay and causing your teeth to become loose.  

Circumscribed scleroderma of the leg

Lung problems

Lung Damage

Almost all people with systemic scleroderma have some loss of lung function. Some people develop severe lung disease, which comes in two forms:

• Pulmonary fibrosis, a hardening or scarring of lung tissue because of excess collagen.
• Pulmonary hypertension, high blood pressure in the artery that carries blood from the heart to the lungs.

Treatment differs for these two conditions:

Localised scleroderma of the face

• Pulmonary fibrosis may be treated with medications that suppress the immune system, including a recently approved kinase inhibitor, which can help counter fibrosis.
• Pulmonary hypertension may be treated with medications that dilate the blood vessels or with newer medications that are prescribed specifically for treating pulmonary hypertension.

To help minimize lung complications, work closely with your doctor.

• Watch for signs of lung disease, including fatigue, shortness of breath, dry cough, or difficulty breathing, and swollen feet. Report these symptoms to your doctor.
• Follow up regularly with your doctor for evaluation of your lung function. This may include standard lung function tests, which measure your lung volumes to monitor the course of lung fibrosis. Checking for pulmonary hypertension early helps doctors manage and treat the condition, even before you may notice symptoms.
• Get regular flu and pneumonia vaccines as recommended by your doctor.

Kidney Problems

• Renal crisis is uncommon but can be serious for people with systemic scleroderma. Renal crisis happens when blood pressure levels rise suddenly to dangerous levels, which can quickly lead to kidney failure. Side effects of certain medications, such as corticosteroids, can also trigger renal crisis. It is important that you and your doctor work together to monitor your blood pressure, including:
• Check your blood pressure regularly, and let your doctor know if you have any new or different symptoms such as a headache or shortness of breath. If your blood pressure is higher than usual, call your doctor right away.
• If you have kidney problems, remember to take your medications as prescribed. In the past two decades, medications known as ACE (angiotensin-converting enzyme) inhibitors have made scleroderma-related kidney failure a less threatening problem than it used to be.

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