CREST syndrome is an autoimmune disease that affects the connective tissues. The word CREST is an acronym for the disease’s five main features.
CREST syndrome affects various systems in the body, and the symptoms a person experiences can depend on the affected organ. Doctors use a variety of tests to diagnose CREST syndrome.
Due to advancements in treatment, the outlook for those with the condition is improving.
This article explores how CREST syndrome affects the body, and its causes, diagnosis, and treatments.
CREST syndrome is a form of systemic sclerosis (SSc), a condition that causes the hardening and tightening of the skin. Other names for this condition are cutaneous systemic sclerosis or limited scleroderma.
In CREST, a person has at least three of the five clinical elements:
- Calcinosis: The deposit of calcium in the skin and other organs.
- Raynaud’s phenomenon: Spasms and narrowing of blood vessels that cause decreased blood flow to fingers and toes.
- Esophageal dysmotility: Difficulty swallowing due to the muscles lining the esophagus losing function.
- Sclerodactyly: Thickening and tightening of the skin on the fingers and toes due to collagen deposition.
- Telangiectasia: Dilated capillaries — a network of small blood vessels — that form red spots on the skin.
CREST syndrome is a type of scleroderma that is slow to progress. Scleroderma refers to a group of diseases affecting the connective tissue that supports the skin and organs.
Doctors consider CREST syndrome a milder form of scleroderma with a better outlook.
Although CREST follows the same process as typical scleroderma, organ involvement comes slower and later in the disease course.
What body systems does scleroderma affect?
In scleroderma, the skin and blood vessels typically become thickened and hard, and people may have swelling or pain in their muscles and joints.
There are 2 main types of scleroderma: localized and systemic.
The localized type only affects the skin, whereas the systemic type affects the skin and other body parts. These might include underlying tissues, blood vessels, and major organs, such as the heart, lungs, and kidneys.
The symptoms of CREST syndrome can vary depending on the affected organs.
Aside from the five clinical elements mentioned above, common symptoms include:
- swelling and stiffness in the joints
- muscle weakness, pain, or stiffness
- swelling or inflammation in areas such as the hands or face
- scarring of the lungs that affects breathing (pulmonary fibrosis)
- heart problems
- kidney problems
Experts are unsure of the exact cause of scleroderma and CREST syndrome. However, it appears to be an autoimmune condition. This means that the immune system attacks the body’s connective tissue.
Several factors likely interact to cause CREST syndrome and scleroderma. These might include:
- atypical immune activity, which affects the way the immune system reacts to body cells
- environmental triggers — scleroderma has associations with silica dust, organic solvents, and L-tryptophan
- genetics, where possessing certain genes may increase the likelihood of having the disease
CREST syndrome does not appear to pass from parent to child.
Various tests, either routine or specialized, can also be beneficial.
The process can include:
- checking the skin for swelling, thickening, and tightening
- blood tests to check for signs of inflammation and autoimmunity
- serum creatinine tests to measure kidney function
- creatinine kinase tests to measure muscle involvement
- imaging tests such as MRI, CT, or X-ray, to assess for any atypical growths
- lung function tests to assess breathing
- electromyography (EMG) and nerve conduction studies
Depending on their symptoms, a person may require a combination of treatments and/or other management strategies.
The primary goals of treatment are to:
- reduce inflammation
- limit the progression of the disease
- improve quality of life
Treatment options may include:
- Anti-inflammatory drugs: Aspirin, indomethacin (Indocin), and naproxen (Naprosyn) can reduce inflammation.
- Immunosuppressants: Steroids may slow disease progression and limit flare-ups.
- D- penicillamine and colchicine: These medications can help control the hardening of the skin and internal organs.
- Vasodilators: These medications ease Raynaud’s symptoms and prevent lung and kidney damage.
- Laxatives or heartburn medication: These medications can reduce digestive symptoms.
People should work with their doctor for individualized treatment plans to control their symptoms. Surgery may sometimes be needed to reduce organ damage or improve blood flow.
People with limited sclerosis may live with pain and discomfort. However, they can often lead normal lives.
However, those with more severe or systemic symptoms may require ongoing monitoring. Severe forms of SSc also have a
The outlook for those with SSc and CREST syndrome is improving. The 10-year survival rate for CREST syndrome, which has slow and late organ involvement, is over 90%.
However, people with SSc have an increased risk of malignancies, especially lung cancer. This means that regular checkups are necessary.
CREST syndrome is a type of scleroderma that affects the skin, small blood vessels, joints, lungs, and other organs.
It can cause symptoms such as Raynaud’s phenomenon, swelling and stiffness in the joints, thickening and hardening of the skin, difficulty swallowing, and other symptoms.
Treatment options vary depending on the severity of the symptoms. However, doctors may prescribe anti-inflammatory and immunosuppressant drugs.
The outlook for those with CREST syndrome is improving due to advances in treatment, and people can often lead normal lives. However, regular checkups are still beneficial because of the increased risk of malignancies.