Scleroderma Treatment in Riverside, CA

Overview of Scleroderma

Scleroderma is an autoimmune disease. It causes swelling and thickening (fibrosis) in the skin and other parts of the body. It starts when the immune system tricks tissues into acting as if they were hurt. This causes swelling, and the body makes too much collagen. Collagen is a protein that gives skin and other tissues their strength. Too much of it leads to tight, hard skin. Scleroderma may affect many parts of your body.

Scleroderma has two main types:

• Localized scleroderma affects only the skin and the tissue just under it.
• Systemic scleroderma, also called systemic sclerosis, affects many parts of the body. It is the more serious type. It can damage your blood vessels and organs, such as the heart, lungs and kidneys. It is split into two kinds, “limited” and “diffuse.” These names tell how much of the skin is affected.

Scleroderma has no cure. The goal of treatment is to ease symptoms and stop the disease from getting worse. It is important to find it early and check it often.

What happens in scleroderma?

No one knows the cause of scleroderma. But researchers think the immune system overreacts. It causes swelling and harm to the cells that line the blood vessels. This makes certain cells, mainly ones called fibroblasts, make too much collagen and other proteins. The fibroblasts also live longer than normal. So collagen builds up in the skin and other organs. This causes some of the signs and symptoms of scleroderma. Blood vessels can also be hurt.

Who Gets Scleroderma?

Anyone can get scleroderma. But some groups have a higher risk. These things may affect your risk:

• Sex. Scleroderma is more common in women than in men.
• Age. It usually starts between ages 30 and 50. It is more common in adults than in children.
• Race. Scleroderma affects people of all races and backgrounds. But it can be more severe in African Americans. It is more common in African Americans than in European Americans. African Americans also tend to get it at a younger age. More of their skin tends to be affected, and they are more likely to have lung disease.

Types of Scleroderma

Localized scleroderma affects the skin and the tissues under it. It is more common in children, but adults can get it, too. It usually shows up in one or both of these patterns:

• Morphea. These are patches of scleroderma. They may be half an inch across or larger.
• Linear scleroderma. The thick skin forms a line. It usually runs down an arm or leg. Sometimes it runs down the forehead and face.

Systemic scleroderma, sometimes called systemic sclerosis, affects your skin, tissues, blood vessels and major organs. Doctors usually split it into two types based on how much skin is affected:

• Limited cutaneous scleroderma. It comes on slowly. It affects the skin on your fingers, hands, face, lower arms and legs below the knees.
• Diffuse cutaneous scleroderma. It comes on faster. At first it affects only the fingers and toes. Then it spreads past the elbows and knees to the upper arms, chest, belly, back or thighs. This type usually does more damage to organs inside the body.

Symptoms of Scleroderma

Symptoms are different for each person. They depend on which type of scleroderma you have.

Localized scleroderma usually causes patches of thick, hard skin in one of two patterns:

• Morphea makes patches of skin turn thick and firm, often in an oval shape. They may look yellow and waxy, with a red or bruise-like edge. The patches may stay in one place or spread to other areas of skin. The disease usually becomes quiet over time. But you may still have dark patches of skin. Some people also feel tired.
• Linear scleroderma causes lines of thick skin, or skin of a different color. They run down your arm or leg, and rarely down the forehead.

Systemic scleroderma, also called systemic sclerosis, may come on fast or slowly. Besides the skin, it may also cause problems with organs inside your body. Many people with this type feel tired.

• Limited cutaneous scleroderma comes on slowly. It usually affects the skin on your fingers, hands, face, lower arms and legs below the knees. It often causes problems with your blood vessels and with your esophagus, the tube that carries food to the stomach. Major organ problems, such as kidney disease or lung disease that gets worse, are less common. It is usually milder than the diffuse form.
• Diffuse cutaneous scleroderma comes on fast. It usually starts with thick skin on your fingers or toes. Then the thick skin spreads to the rest of your body above the elbows, knees or both. This type can damage organs inside your body, such as any part of your digestive system, your lungs, your kidneys and your heart.

Causes of Scleroderma

Researchers do not know the exact cause of scleroderma. But they think several things may lead to it:

• Genes. Genes can raise the chance that a person gets scleroderma. They also help decide which type they get. But you cannot inherit the disease. It is not passed from parent to child like some gene diseases. Still, parents, children, brothers and sisters of people with scleroderma have a higher risk than most people.
• The world around you. Researchers think contact with some things, such as some chemicals, may set off scleroderma.
• Changes in the immune system. Unusual activity of the immune system causes changes in cells. These changes lead to too much collagen. In some cases, the immune system’s reaction to cancer cells that are starting to grow may set off scleroderma.
• Hormones. Women get most types of scleroderma more often than men. Researchers think differences in hormones or in the immune system between women and men may play a part.

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Diagnosis of Scleroderma

Scleroderma can be hard to diagnose. Symptoms are different for each person. They are also like those of other diseases. No single test can show that you have it. Instead, doctors use a mix of steps. Your doctor may:

• Ask about your health history.
• Ask about your symptoms now and in the past.
• Give you a physical exam.

Your doctor may suggest more tests, such as:

• Blood tests for certain antibodies that attack your own tissues by mistake. Some of these antibodies are common in people with scleroderma. But other things can cause them too. So a blood test alone cannot show that you have scleroderma.
• A skin biopsy. A tiny piece of skin is checked under a microscope.

Your doctor may order more tests to look for problems with organs inside your body, such as the heart, lungs or kidneys. Finding organ problems early helps doctors treat and manage the disease. Tests may include:

• A CT scan, which takes pictures of the lungs and other organs.
• An echocardiogram, which uses sound waves to make moving pictures of your heart.
• Lung function tests, which show how well your lungs work.

Treatment of Scleroderma

Your treatment depends on which type of scleroderma you have, your symptoms, and which tissues and organs are affected. Treatment can help control symptoms and limit damage.

Your doctor may suggest medicines such as:

• Anti-inflammatory medicines to ease pain and swelling.
• Creams to treat skin changes, such as tightness and itching.
• Immunosuppressants. They may calm an overactive immune system and help control some parts of the disease. You may take them by mouth, through an IV or as a shot.
• Vasodilators. They help blood vessels widen. They may treat Raynaud’s phenomenon and some lung problems.

Your doctor may also prescribe medicines that are usually approved for other rheumatic diseases with symptoms like those of scleroderma.

Many people get help from physical or occupational therapy. It can:

• Ease pain.
• Make muscles stronger and help them move better, including the muscles in your arms, legs and jaw.
• Teach you ways to do daily tasks. For example, if hand pain and stiffness make it hard to brush your teeth, a therapist can suggest toothbrushes and tools that make flossing easier.

Regular dental care is important. Scleroderma can make your mouth dry. It can also damage the tissues in your mouth. This can speed up tooth decay and make your teeth loose. Tight skin on the face can also make your mouth opening smaller. That makes it harder to care for your teeth. Here are some ways to avoid tooth and gum problems:

• Brush and floss your teeth regularly.
• Get regular dental checkups. Call your dentist right away if you have mouth sores, mouth pain or loose teeth.
• Talk to your dentist and doctor about the best ways to keep your mouth moist.
• Use special mouthwash or toothpaste for dry mouth. You can also ask your doctor about medicines for dry mouth.

Lung Damage

Almost all people with systemic scleroderma lose some lung function. Some people get severe lung disease. It comes in two forms:

• Pulmonary fibrosis. The lung tissue becomes hard and scarred from too much collagen.
• Pulmonary hypertension. This is high blood pressure in the artery that carries blood from the heart to the lungs.

Each one is treated differently:

• Pulmonary fibrosis may be treated with medicines that calm the immune system or that help fight the scarring.
• Pulmonary hypertension may be treated with medicines that widen the blood vessels.

Work closely with your doctor to lower your risk of lung problems:

• Watch for signs of lung disease. These include feeling tired, shortness of breath, a dry cough, trouble breathing and swollen feet. Tell your doctor about these symptoms.
• See your doctor regularly to check how well your lungs work. This may include lung function tests. They measure how much air your lungs hold, to track lung scarring over time. Checking for pulmonary hypertension early helps doctors treat it, even before you notice symptoms.
• Get flu and pneumonia shots as your doctor advises. This is most important if you take medicines that calm the immune system or if you have lung disease.

Heart Problems

Some people get heart problems, such as:

• Cardiomyopathy. The heart becomes scarred and weak.
• Myocarditis. The heart muscle becomes swollen.
• Arrhythmia. The heart beats in an uneven way.

Treatment for heart problems depends on the problem. It can range from medicine to surgery.

Kidney Problems

Renal crisis is not common. But it can be serious for people with systemic scleroderma. It happens when blood pressure suddenly rises to a dangerous level. This can quickly lead to kidney failure. Side effects of some medicines, such as corticosteroids, can also set off renal crisis. Work with your doctor to watch your blood pressure:

• Check your blood pressure regularly. Tell your doctor about 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, take your medicines just as your doctor prescribed. Over the past several decades, medicines called ACE inhibitors (angiotensin-converting enzyme inhibitors) have made kidney failure from scleroderma much less of a threat than it used to be.

Who Treats Scleroderma?

Most people see a rheumatologist for scleroderma. A rheumatologist is a doctor who is an expert in rheumatic diseases. These include arthritis and other diseases of inflammation or the immune system. Dermatologists treat problems with the skin, hair and nails. They may also play an important part, mostly for people with localized scleroderma.

Scleroderma can affect many organs. So you may have several doctors caring for you. They may include:

• Cardiologists, who treat diseases of the heart and blood vessels.
• Dental providers. They can treat problems caused by thick tissue in the mouth and face.
• Gastroenterologists, who treat stomach and gut problems.
• Mental health professionals. They offer counseling and treat problems such as depression and anxiety.
• Nephrologists, who treat kidney disease.
• Occupational therapists, who teach you how to do daily tasks safely.
• Orthopaedists, who treat bone and joint diseases and injuries and do surgery on them.
• Primary care providers, such as doctors, nurse practitioners and physician assistants.
• Physical therapists, who teach you ways to build muscle strength.
• Pulmonologists, who treat lung problems.
• Speech-language pathologists. They treat problems with speech, communication and swallowing.

Living With Scleroderma

Living with scleroderma may be hard. It depends on which type you have and your symptoms. Try to take an active part in your treatment. These tips may help:

• Keep warm. Your body controls its temperature through the skin. So dress in layers, wear gloves and socks, and stay out of cold rooms and weather when you can.
• Try to stay away from cold or wet places that may set off Raynaud’s phenomenon.
• If you smoke, quit. Nicotine and smoking make blood vessels tighten. This can make some symptoms worse and cause lung problems.
• Put on sunscreen before you go outside, to protect your skin from more sun damage.
• Use lotion on your skin to help ease stiffness.
• Use humidifiers to add moisture to the air in your home in cold winter weather. Clean them often so germs do not grow in the water.
• Do not take hot baths or showers. Hot water dries the skin.
• Stay away from harsh soaps, household cleaners and strong chemicals. Wear rubber gloves if you use them.
• Exercise regularly. Exercise, mostly swimming, helps blood flow to the areas that are affected.
• See the dentist regularly for checkups.
• Join support groups online or in your area.
• Keep talking. Talk to your family and friends to help them understand the disease.
• Talk to a mental health professional for help coping with a long-term illness.

Some types of scleroderma can affect parts of the digestive system. Doctors may prescribe medicines for heartburn, constipation and slow movement of food through the gut. These tips may help if you have stomach or gut symptoms:

• Eat small meals often.
• Stay upright for 3 hours after meals. Try not to lie back or slouch after eating.
• Eat moist, soft foods, and chew them well. If you have trouble swallowing, or your body does not take in nutrients well, your doctor may prescribe a special diet.
• Drink less alcohol and caffeine.
• Drink plenty of water.
• When it is time to sleep, raise the head of your bed with blocks, or use a wedge pillow. A stack of pillows does not help as much as raising the head of the bed with blocks or wedges.

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