Scleroderma can cause different symptoms in each person. It can affect just the skin, or it may damage internal organs such as the heart, lungs and kidneys. It can also cause complications like Raynaud’s phenomenon, gastrointestinal problems and severe fatigue.
Doctors don’t know what causes scleroderma, but it’s thought that the immune system becomes overactive. This causes cells to produce too much collagen.
What is Scleroderma?
Scleroderma is an autoimmune disease that occurs when the immune system makes too much collagen, a tough tissue that supports blood vessels and internal organs. The extra collagen causes the skin and other tissues to thicken, or harden. Scleroderma can be limited to just the skin, or it may affect the underlying muscles and bones. It can also cause inflammation and damage to blood vessels. The condition can be life-threatening if it causes problems with the heart, lungs, or kidneys.
Doctors don’t know what causes scleroderma. But they think it’s an inherited disorder that can be triggered by changes in the environment or genes. Scleroderma usually develops in people between 30 and 50. It’s four times more common in women than men. It’s also more common in people who were assigned female at birth (AFAB). This may be because of different hormones that affect the chance of getting scleroderma and the severity of it.
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In some types of scleroderma, the hardening and tightening of the skin is more limited. The two main forms of this type are morphea scleroderma and linear scleroderma. The symptoms of morphea scleroderma usually improve or go away on their own. Linear scleroderma usually starts on the arms or legs. It can cause skin ulcers and a tight, waxy feeling skin. This form of the condition is sometimes called sclerodactyly or Raynaud’s phenomenon. It can lead to a decrease in the ability to bend the fingers and toes, or to make fists. It can also cause joint pain, muscle weakness, and kidney problems.
The treatment for scleroderma depends on the type and severity of the condition. Doctors may prescribe medicines to ease symptoms. These include steroid creams for the skin, and medicines that help with breathing and other symptoms caused by the underlying muscle or organ involvement. In some cases, doctors will recommend physical therapy to help retrain muscles and joints. They can also suggest aids and equipment to help people with scleroderma do daily activities, such as using door handles and eating. Doctors can also help manage complications of scleroderma, such as kidney failure and gastrointestinal issues.
Symptoms
Scleroderma can affect the skin, joints, lungs and other organs. The type of scleroderma you have and where it spreads to your body will determine what symptoms you get. It can be localized (limited cutaneous), which comes on slowly and affects the skin on your fingers, hands, face and knees, or it can be widespread (diffuse cutaneous systemic sclerosis). This form of scleroderma may cause internal organ damage such as high blood pressure in the legs and feet, excess fluid buildup around the heart (pericardial effusion), kidney disease, lung complications and stomach problems.
The skin problems caused by scleroderma include thick, discoloured patches of skin that become firm and hard. They are usually in one of two patterns: a reddish or purple area with a waxy appearance called erythema nodosum and a line or band of different-coloured skin that runs down the arm, leg or, less commonly, on the forehead. Some people with scleroderma develop Raynaud’s phenomenon, an exaggerated response to cold temperatures or emotional stress that causes the fingers and toes to turn white or blue. Pain, stiffness and swelling of the joints can also occur. These are due to fibrosis which occurs around the tissue and tendons of joints. Some people with scleroderma have a condition called calcinosis, which is when mineral crystal deposits (calcium sulfate) form under the skin and make small, hard lumps that look like scars or ulcers.
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People with scleroderma are often very tired and do not feel well. This is because the immune system attacks healthy tissues and organs, causing inflammation. This can lead to long-term damage, causing the symptoms of scleroderma.
To diagnose scleroderma, your doctor will ask about your symptoms and do a physical exam. He or she may also order blood tests to check your level of certain antibodies and a sample of skin to be tested in a lab. Your doctor will also check your lungs, heart and abdomen to look for signs of the disease. A CT scan — a type of x-ray that produces detailed images of your bones, soft tissues and blood vessels — may be done to check for problems in the lungs and other organs.
Diagnosis
Scleroderma can be hard to diagnose, as it can affect different parts of the body and symptoms can come and go. It is usually a chronic condition that requires long-term treatment. It can lead to serious and life-threatening complications if it affects the tissue inside organs such as the heart, lungs or esophagus. It may also cause kidney disease or damage to the blood vessels and tendons.
It is thought that scleroderma occurs when an abnormal part of the immune system attacks and destroys blood vessels in the skin and internal organs. This causes the blood to produce too much collagen, a tough protein that makes scar tissue form. When normal tissue from the skin, lungs or blood vessels is replaced by this scar-like tissue, these body parts can’t work as well. It’s not clear what causes this to happen, but certain genes increase your risk.
A doctor can find some signs of scleroderma, such as Raynaud’s phenomenon or an unusual pattern of small blood vessels at the base of the fingernails (called the salt and pepper sign). They can also check for other problems that can develop with scleroderma, such as ulcers in the mouth or gastrointestinal bleeding.
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The two main types of scleroderma are limited and systemic scleroderma. With limited scleroderma, the disease usually affects only one part of the body and does not spread to other areas. People with this type of scleroderma are less likely to develop complications, such as a problem with the joints or pulmonary hypertension (high blood pressure in the lungs).
In the more common systemic scleroderma, the disease can spread quickly and can affect several organs, such as the kidneys, lungs, heart, intestines, muscles and bones. This type of scleroderma is more likely to lead to severe complications and can be fatal.
Your doctor can recommend medications to control your symptoms. These may include medicines that suppress the immune system, medicines to reduce inflammation and pain relievers. Medicines to help with digestion, such as pills to reduce stomach acid and medicines to make food move through the intestines, can also be helpful. People with systemic scleroderma should get regular blood tests to watch for signs of kidney disease, such as high levels of protein in the urine.
Treatment
Although no medicine can stop the body from making too much collagen, several drugs can control symptoms and prevent complications. They can ease or eliminate symptoms of localized scleroderma and prevent or delay the progression of systemic scleroderma to other organ systems, such as the kidneys, heart, lungs, or digestive tract.
Scleroderma may improve on its own, but the thickened skin and other organ damage will be lifelong (permanent). A healthcare provider will perform tests to diagnose scleroderma and check for problems with your heart, lungs, or gastrointestinal tract. These include blood tests to see how well your immune system is working; a lung function test or chest X-ray to measure the amount of fluid in your lungs; and an endoscopy or biopsy to take a small sample of tissue for testing.
The cause of scleroderma is unknown, but it appears to be an autoimmune disease, in which your immune system mistakenly attacks healthy tissue in the body and causes too much collagen to build up. In localized scleroderma, this usually affects the skin, but in some types such as linear scleroderma and morphoea it can also affect muscles, bones, internal organs, and blood vessels.
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There is no cure for scleroderma, but several treatments can ease or eliminate your symptoms and help you maintain your normal life activities. Medications may treat dry skin, ease pain, and slow or reverse complications. They can also treat the inflammation that causes scleroderma, and prevent or reduce problems with your lungs, heart, and kidneys.
Medications that open your blood vessels help to treat Raynaud’s phenomenon and control blood pressure. Other medications reduce inflammation and block damaging immune factors.
Physical therapy can teach you exercises to keep your joints and muscles flexible and help you manage your pain. Other treatment options include hydration, warm baths, and moisturizing creams. Special gels can help you urinate more easily, and hyaluronidase injections can treat the condition that makes it hard to open your mouth (microstomia). Steroid creams or pills can also be used to reduce pain and inflammation.
