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CREST syndrome , also known as limited scleroderma, is a widespread connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. The symptoms involved in CREST syndrome are associated with the generalized form of the disease systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease.
(C) - Calcinosis: calcium deposits in the connective tissues
(R) - Raynaud's phenomenon: where the hands and feet turn white and cold and then blue, in response to cold or anxiety
(E) - Esophageal dysfunction resulting in swallowing difficulty
(S) - Sclerodactyly: thick and tight skin on the fingers, caused by an excess of collagen deposits within skin layers.
(T) - Telangiectasia: small red spots on the hands and face that are caused by the swelling of tiny blood vessels.
At least two of these five features need to be present to be diagnosed with the disease. CREST syndrome is believed to be an autoimmune disorder, where the immune system appears to stimulate the production of too much collagen which builds up in the skin and internal organs, impairing their function. There is no cure. Current treatment focuses on relieving signs and symptoms and preventing complications.
Source: GARD Last updated on 05-01-20
The immune system in people with CREST syndrome appears to stimulate cells called fibroblasts to produce excess amounts of collagen. The hallmark of scleroderma is progressive fibrosis of tissues. Normally, fibroblasts make collagen to help heal wounds, but in this case, the protein is produced even when it's not needed, forming thick bands of connective tissue around the cells of the skin, blood vessels and in some cases, the internal organs.
Although an abnormal immune system response and the resulting production of excess collagen appears to be the main cause of limited scleroderma, researchers suspect that other factors increase the risk to have the disease, including:
Last updated on 05-01-20
CREST syndrome can be difficult to diagnose. Signs and symptoms vary widely and often resemble those of other connective tissue and autoimmune diseases. Further complicating matters is that limited scleroderma sometimes occurs with other autoimmune conditions — such as polymyositis, lupus and rheumatoid arthritis.
Diagnosis is usually based in the typical symptoms (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). People with CREST syndrome usually present at least three of these five main clinical features, but the diagnosis can be made when only two these five features are present. Other exams that might help to make a diagnosis include:
Last updated on 05-01-20
Unfortunately, CREST syndrome has no known cure. Treatment generally focuses on relieving symptoms and preventing complications:
Calcinosis : No treatment seems to be available to prevent or eliminate calcinosis. Large or painful calcium deposits sometimes need to be surgically removed, and amputation of fingertips may be necessary if the skin ulcers progress to gangrene. One or a combination of the following treatments may be tried on a case-by-case basis; however, larger studies are needed to prove efficacy.
Raynaud phenomenon : The following treatment is suggested for the treatment of people with Raynaud phenomenon:
Esophageal dysmotility : The treatment of esophageal dysmotility and gastroesophageal reflux in people with scleroderma is the same as in patients without scleroderma. Treatment involves behavior changes, medication (H2 blockers), and a surgical procedure (esophageal dilatation) in more severe cases. Esophageal dilatation can help when severe feeding difficulty or regurgitation occur due to an esophageal narrowing.
Sclerodactyly : Treatments include medication (corticosteroids, nonsteroidal anti-inflammatory drugs, D-penicillamine, IFN-gamma, cyclosporine, and cytostatic drugs). Skin involvement in CREST syndrome typically is not severe; therefore, treatment is not need in many cases.
Telangiectasia : Pulsed-dye laser treatment seems effective for the treatment the telangiectasia in the face, but this has not been specifically studied in CREST patients. Treatment may include estrogen-progesterone or desmopressin, laser ablation, or sclerotherapy.
Other treatment may include stretching exercises for the finger joints, and facial exercises that may help keep the face and mouth flexible, under a physical therapist supervision. If CREST syndrome is making it difficult to do daily tasks, an occupational therapist can help individuals learn new ways of doing things. For example, special toothbrushes and flossing devices can make it easier to care for the teeth. Surgery may be necessary for some affected individuals.
About 45% of people with systemic sclerosis may have depression and 64% also develop anxiety, so it is recommended to have an early assessment and treatment..
For pain management, studies have shown that oxycodone is effective and safe for pain due to severe skin ulcers, while topical lidocaine helps reduce pain of the finger's ulcers in people with systemic scleroderma.
There are also some lifestyle changes and home remedies that may be helpful for some people with CREST syndrome. For example:
The information provided here is for general educational purposes only. Individuals interested in learning about specific treatment options for themselves or family members should speak with their doctors.
Last updated on 05-01-20
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