What Is CREST Syndrome?
Scleroderma is a rare, autoimmune disease in which normal and healthy tissue is replaced with thick fibrous tissue that leads to hardening and tightening of the skin. Limited scleroderma is one of its types known as CREST syndrome. CREST syndrome most often affects the skin and other body parts, including the gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles, and joints.
What Does CREST Stand For?
Each letter in the word CREST stands for a feature of the disease:
- Calcinosis (calcium lumps in the skin).
- Raynaud's phenomenon (cold skin when exposed to cold or when stressed).
- Esophageal dysmotility (difficulty swallowing).
- Sclerodactyly (thickening and tightening of the skin).
- Telangiectasias (red spots on the skin).
What Causes CREST Syndrome?
The exact cause of CREST syndrome or limited scleroderma is unknown. Rarely CREST syndrome runs in families, and most cases do not show any family history of the disease. Also, it is not contagious.
What Are the Symptoms of CREST Syndrome?
Usually, People with CREST syndrome have at least two of the five symptoms:
Calcinosis: Abnormal calcium lumps in the fingers or other body parts.
Raynaud's Phenomenon: White, blue or cold, painful and numb skin, particularly on the hands and feet when exposed to cold or stress. It is caused by inappropriate contraction of the blood vessels in the fingers and toes in response to complex or emotional distress.
Esophageal Dysfunction: People with this condition have difficulty swallowing due to scarring in the esophagus.
Sclerodactyly: Tightness and thickening of the skin. The most commonly affected parts are the fingers, feet, hands, and face. The skin thickening can also involve other sites such as the forearms, upper arms, chest, abdomen, lower legs, and thighs.
Telangiectasias: Some people may also experience red spots on the hands, palms, forearms, face, and lips. The widening of blood vessels causes these spots.
What Are the Risk Factors for CREST Syndrome?
Several combined factors influence the risk of developing CREST syndrome:
Genetics: People with certain gene variations are more likely to develop CREST syndrome or other forms of scleroderma.
Environment: Past researches suggest that in some people, symptoms may be triggered by exposure to certain viruses, medications, drugs, or certain harmful substances or chemicals at work.
Immune System: Since CREST syndrome is believed to be an autoimmune disease, People living with this condition also have symptoms of other autoimmune disorders such as rheumatoid arthritis, lupus, or Sjogren's syndrome.
What Are the Complications of CREST Syndrome?
The complications can affect the different body parts:
Fingertips: In some cases, Raynaud's phenomenon can be so severe that the restricted blood flow damages the tissues at the fingertips, causing pits or skin sores.
Teeth: Severe tightening of facial skin can make the mouth smaller and narrower, which may lead to difficulty brushing the teeth. In addition, people may not produce average amounts of saliva, thus increasing the risk of tooth decay.
Digestive System: Digestive problems associated with CREST syndrome can lead to heartburn and difficulty swallowing. It can also cause cramps, bloating, constipation or diarrhea.
Joints: The tightening of the skin over joints can limit joint flexibility, particularly in the hands.
How Is CREST Syndrome Diagnosed?
Since different body parts are affected, it can be challenging to diagnose CREST syndrome.
Gastrointestinal Tests: CREST syndrome can affect esophageal muscles and intestinal walls, which can cause heartburn, difficulty in swallowing and absorption of nutrients, and movement of food through the intestine. The doctor may perform an endoscopy (inserting a small tube with a camera on the end) to view the esophagus and the intestines.
Blood Tests: Blood tests help check for elevated levels of immune factors, known as antinuclear antibodies.
How Is CREST Syndrome Treated?
Treatment involves the management of symptoms:
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Topical medications are beneficial. Moisturizers help keep the skin moist and treat hardened skin.
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Blood pressure medications help dilate blood to treat Raynaud's phenomenon.
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Antibiotics and medicines that help move food through the intestines may help reduce bloating, diarrhea, and constipation. In addition, over-the-counter and prescribed antacids and proton pump inhibitors help people with heartburn and other digestive difficulties.
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Anti-inflammatory drugs are beneficial for people who have joint problems. These drugs reduce inflammation and hence the pain and swelling.
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Immunosuppressive medicines.
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Physical therapy that prevents joints from contracting can sometimes be helpful. It can help improve strength and mobility.
General Management
Specific steps can help manage your symptoms of CREST syndrome. They are:
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Exercise enhances the body's flexibility and relieves stiffness. Regular exercises or physical activities can help keep the skin and joints flexible.
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Take good care of dry or stiff skin by regularly applying soaps and creams designed especially for dry skin. Avoid hot water baths and showers and exposure to chemicals or harmful substances, irritating and drying the skin.
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Quit smoking as nicotine causes blood vessels to contract, making Raynaud's disease worse. Smoking can also lead to permanent narrowing of the blood vessels.
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Avoid foods that cause heartburn or gas. Antacids may help relieve symptoms. Avoid late-night meals and elevate the head of the bed to keep stomach acid from backing up into the esophagus. Drinking enough water and eating fibrous foods help avoid constipation.
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Protect the body from the cold by wearing layers of warm clothes and covering the face and head. Keeping the body temperature warm is also essential to help prevent Raynaud's symptoms. In addition, wear loose-fitting shoes to keep the blood supply moving to your feet.
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Avoid injury to joints by not lifting heavy objects that may place a strain on them, thus risking further injury.
Conclusion
CREST syndrome affects the skin and many other body parts. Although no specific cure is available for the disorder, it is manageable, and people living with this disease may still lead healthy and productive lives. However, like many other conditions, proper education about the disease can be crucial in managing it and reducing the risk of further complications.
