Description

Scleroderma (sklair-oh-DUR-muh), also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin. Scleroderma can also cause problems in the blood vessels, internal organs and the digestive tract.

Scleroderma is often classified as limited or diffuse, which refers only to the extent of skin involvement. Both types can involve any of the other vascular or organ of the symptoms that are part of the disease. Localized scleroderma, also known as morphea, only affects the skin.

While there is no cure for scleroderma, treatments can relieve symptoms, slow the progression and improve the quality of life.

Symptoms

The symptoms of scleroderma vary from person to person, depending on what parts of the body are affected.

Skin-related symptoms

Almost all those who have scleroderma experiences of setting and hardening of the skin.

The first parts of the body that are affected are usually the fingers, hands, feet and face. In some people, the thickening of the skin may also involve the forearms, arms, chest, abdomen, legs and thighs. The first symptoms may include swelling and itching. The color of the affected skin may be lighter or darker, and the skin may appear shiny because of the oppression.

Some people also have small red spots, called telangiectasia, on his hands and face. Calcium deposits can form under the skin, especially on the tip of the fingers, causing bumps that can be seen on x-rays.

Raynaud's phenomenon

Raynaud's phenomenon is common in patients with scleroderma. This happens due to an exaggerated contraction of the small blood vessels in the fingers and toes in response to cold temperatures or emotional distress. When this happens, the digits may feel pain or numbness and turn white, blue, gray, or red. Raynaud's phenomenon can also occur in people who do not have scleroderma.

Digestive symptoms

Scleroderma can affect any part of the digestive tract, from the esophagus to the rectum. Depending on what parts of the digestive system are affected, symptoms may include:

  • Heartburn.
  • Difficulty swallowing.
  • The abdominal distension.
  • The diarrhea.
  • The constipation.
  • The Fecal incontinence.

Heart - and lung-related symptoms

When scleroderma affects the heart or the lungs, may cause shortness of breath, decreased exercise tolerance, and dizziness. Scleroderma can cause scarring in the tissues of the lung may result in the increase of the lack of air over time. There are medications that can help slow the progression of this disease to the lung.

Scleroderma can also cause an increase in blood pressure in the circulation between the heart and the lungs. This is called pulmonary hypertension. In addition to causing shortness of breath, pulmonary hypertension can also cause excessive accumulation of fluid in the legs, feet, and, sometimes, around the heart.

When scleroderma affects the heart, the heartbeat can become irregular. Heart failure can also happen in some people.

Causes

Scleroderma occurs when the body produces too much collagen and accumulates in the tissues of the body. Collagen is a fibrous type of protein that causes the body's connective tissues, including the skin.

Experts do not know exactly what causes this process to begin, but the body's immune system seems to play a role. Most likely, the scleroderma is caused by a combination of factors, including immune system problems, the genetic and the environmental factors that trigger.

Risk factors

Any person can develop scleroderma, but it is more common in people assigned female at birth. People often get the scleroderma between the ages of 30 and 50. Blacks tend to have an earlier onset and are more likely to have involvement of the skin and the lungs.

Several other factors combined to appear to influence the risk of scleroderma:

  • Genetics. People who have certain changes in the genes seem to be more likely to develop scleroderma. This may explain why the scleroderma seems to run in families in a small number of people and why some types of scleroderma are more common for individuals in certain racial and ethnic groups.
  • The environmental triggers. The research suggests that in some people, the symptoms of scleroderma can be triggered by exposure to certain viruses, medications, or drugs. The repeated exposure, such as at work, in the certain harmful substances or chemicals can also increase the risk of scleroderma. An environmental trigger is not identified by the majority of the people.
  • Immune system conditions. Scleroderma is believed to be an autoimmune disease. This means that it is produced, in part, because the body's immune system begins to attack the connective tissues. People who have scleroderma may also have symptoms of other autoimmune disease such as rheumatoid arthritis, lupus or Sjogren's syndrome.

Complications

Scleroderma complications ranging from mild to severe and can affect the:

  • The tips of the fingers. In systemic sclerosis, Raynaud's phenomenon may be so severe that the restricted blood flow permanently damages the tissue at the tip of the fingers, causing wells or sores on the skin. In some people, the tissue at the tip of the fingers can die.
  • Lungs. The scarring of the lung tissue can affect the ability to breathe and exercise tolerance. High blood pressure in the arteries of the lungs may also occur.
  • Kidneys. A serious complication of kidney, called scleroderma renal crisis, implies a sudden increase in blood pressure and rapid kidney failure. The rapid treatment of this condition is important to preserve renal function.
  • Heart. The healing of the tissues of the heart, increases the risk of irregular heartbeat and heart failure. Scleroderma can also cause inflammation of the sac surrounding the heart.
  • Teeth. Severe hardening of the skin of the face can cause the mouth to be smaller and narrower. This can make it difficult to brush your teeth or have them professionally cleaned or restored. People who have scleroderma often do not do typical amounts of saliva, so that the risk of dental caries increases even more.
  • The digestive system. Digestive complications of scleroderma may include heartburn, and difficulty swallowing. Scleroderma can also cause attacks of cramps, bloating, constipation or diarrhea. Some people who have scleroderma may also have problems absorbing nutrients due to the excessive growth of bacteria in the gut.
  • In the joints. The skin over the joints can become so strong that it restricts the flexibility and the movement, especially in the hands.

Diagnosis

Because scleroderma can take many forms and affects many different areas of the body, it can be difficult to diagnose.

After a thorough physical exam, your health care professional may suggest a blood test to detect elevated levels of certain antibodies produced by the immune system.

Your healthcare provider may also suggest other tests of blood, or of images or of an organ function tests. These tests can help determine if your digestive system, the heart, lungs, or kidneys are affected.

Treatment

There is no treatment that can cure or stop the overproduction of collagen that occurs in scleroderma. But a variety of treatments that can help control symptoms and prevent complications.

Medications

Because scleroderma can affect many different parts of the body, the choice of the medication varies depending on the symptoms. Examples include drugs that:

  • Dilate the blood vessels. The blood pressure medications that can dilate the blood vessels, can help treat Raynaud's phenomenon.
  • Suppress the immune system. Medications that suppress the immune system, such as those taken after organ transplants, can help to reduce the progression of some of the symptoms of scleroderma, such as the thickening of the skin or deterioration of lung damage.
  • Reduce digestive symptoms. Pills to reduce the stomach acid can help relieve heartburn. Antibiotics and medications that help to move food through the intestines can help reduce the bloating, diarrhea and constipation.
  • Prevent infections. It is recommended that vaccines are important to protect people with scleroderma of infectious diseases. Talk with your healthcare provider about the vaccines for flu, pneumonia, herpes, HPV, COVID-19, and RSV.
  • Relieve the pain. If the pain medicine available without a prescription does not help enough, your health care professional may be able to suggest the prescription of medication to control the pain.

Therapies

Physical therapists, occupational therapists can help improve your strength and mobility and to maintain independence with daily tasks. Hand therapy can help prevent stiffness, also called contractures.

Surgical and other procedures

Stem cell transplants may be an option for people who have severe symptoms that have not responded to more common treatments. If the lungs or kidneys have been severely damaged, organ transplants could be considered.

Lifestyle and home remedies

You can take a series of measures to help manage the symptoms of scleroderma:

  • Stay active. Exercise keeps the body flexible, improves circulation and relieves stiffness. The range-of-motion exercises that can help to maintain the skin and flexible joints. This is always very important, especially early in the course of the disease.
  • Protect your skin. Take good care of dry or hard skin through the use of lotion, and a sunscreen regularly. Avoid hot baths and showers, and exposure to harsh soaps and household chemicals, which can irritate and dry your skin.
  • Do not smoke. Nicotine causes the blood vessels to constrict, making the phenomenon of Raynaud's worse. Smoking can also cause permanent narrowing of the blood vessels and cause or worsen the problems of the lungs. Quitting smoking can be difficult. Ask your health professional for help.
  • Control the acidity. Avoid foods that give you heartburn or gas. Also avoid the late-night meals. Elevate the head of the bed to keep the stomach acid up into the esophagus of sleep. Antacids can help alleviate the symptoms.
  • Protect yourself from the cold. Wear warm gloves in any time that your hands are exposed to cold — even when you come to a freezer. It is also important to keep the core temperature of the hot body to help prevent Raynaud's phenomenon. When you're out in the cold, wear warm boots, cover your face and head, and wear layers of warm clothing.

Coping and support

As with other chronic diseases, living with scleroderma can cause you to feel anxious or worried. Here are some ideas to help you even your feelings:

  • Keep your typical daily activities in the best possible way.
  • Pace yourself and make sure you get the rest you need.
  • Stay connected with friends and family.
  • Continue hobbies that you like to and are able to do.

Keep in mind that your physical health can have a direct impact on your mental health. People with chronic illnesses can feel the rejection, anger, and frustration.

Sometimes, you may need more tools to deal with their emotions. Mental health professionals, such as therapists or the behavior of psychologists, may be able to help you put things in perspective. It can also help you develop coping skills, including relaxation techniques.

Join a support group where you can share experiences and feelings with other people, it is often a good approach. Ask your health care team what support groups are available in your community.

Preparing for your appointment

Probably the first to bring your symptoms to the attention of the family health care professional. You may be referred to a doctor who specializes in the treatment of arthritis and other diseases of the joints, muscles, and bones. This type of doctor is called a rheumatologist. Because scleroderma can affect many organs and systems, you may need to see a variety of medical specialists.

What you can do

Time with their health care professionals may be brief. To make the best use of the limited time, plan ahead and write lists of important information, such as:

  • Detailed descriptions of all of your symptoms.
  • A list of all your medicines and supplements, including the dose.
  • Questions for the health care professional, such as what tests or treatments that you may need.

What to expect from your doctor

Your health care professional may do some of the following questions:

  • Make your fingers change colour when you are cold?
  • Generally, the experience heartburn or difficulty in swallowing?
  • Have you noticed any stretching of the skin or thickening of the skin?
Symptoms and treatment of Scleroderma