Bay Biosciences provides high quality, clinical grade bio-samples, cryogenically preserved biopsy tissue, FFPE blocks, matched sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with CREST syndrome disease.
The cryogenically preserved sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patient’s peripheral whole-blood using customized processing protocols. The CREST syndrome bio-specimens are collected from unique patients diagnosed with CREST syndrome and are provided to a valued pharmaceutical customer for research, development and drug discovery.
CREST Syndrome
CREST syndrome, also known as Limited Scleroderma (sub type of Scleroderma) or limited cutaneous form of Systemic Sclerosis (LcSSc), is a multi-system connective tissue disorder. CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features of the syndrome.
The acronym “CREST” refers to the five main features: Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly and Teangiectasia.
The skin changes (hardened skin) associated with limited scleroderma typically occur only in the lower arms and legs, below the elbows and knees, and sometimes affect the face and neck. Limited scleroderma can also affect the digestive tract, heart, lungs or kidneys. The problems caused by limited scleroderma may be minor. Sometimes, however, the disease affects the lungs or heart, with potentially serious results. CREST syndrome or limited scleroderma has no known cure. Treatments focus on managing symptoms, preventing serious complications and improving quality of life.
Signs & Symptoms of CREST Syndrome
While some varieties of scleroderma occur rapidly, signs and symptoms of CREST Syndrome or limited scleroderma usually develop gradually. They include:
- Tight, hardened skin- In CREST syndrome or limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.
- Raynaud’s Phenomena- This condition occurs when small blood vessels in the fingers and toes spasm in response to cold or emotional stress, blocking the flow of blood. In most people, the skin turns white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and might throb or tingle. Raynaud’s phenomena is often the first sign of CREST syndrome or limited scleroderma, but many people who have Raynaud’s never develop scleroderma.
- Red spots or lines on skin. The swelling of tiny blood vessels near the skin’s surface cause these small red spots or lines (telangiectasias). These red spots on the skin are not painful, they occur primarily on the hands and face.
- Bumps under the skin. CREST syndrome or limited scleroderma can cause tiny calcium deposits (calcinosis) to develop under the skin, mainly on the elbows, knees and fingers. You can see and feel these deposits, which sometimes are tender or become infected.
- Swallowing difficulties- CREST Syndrome or limited scleroderma commonly causes problems with the tube that connects the mouth and stomach (esophagus). Poor functioning of the muscles in the upper and lower esophagus can make swallowing difficult and allow stomach acids to back up into the esophagus, leading to heartburn, inflammation and scarring of esophageal tissues.
Causes of CREST Syndrome
The causes of CREST syndrome are not known, but CREST syndrome or limited scleroderma is believed to be an autoimmune disorder, in which the patients immune system turns against their own body. The immune system appears to stimulate the production of too much collagen, a key component of connective tissue. This overproduction of collagen builds up in the skin and internal organs so that they don’t function normally.
Risk factors of CREST Syndrome
- Patients Sex- Women are far more likely to develop CREST syndrome or limited scleroderma than men.
- Age- CREST syndrome or limited scleroderma is more common between the ages of 30 and 50.
- Race- In the United States, limited scleroderma tends to be more severe in blacks and native Americans than in Caucasians.
- Genetic factors- If someone in the family has an autoimmune disease, such as lupus, rheumatoid arthritis or Hashimoto’s disease they have an increased risk of developing CREST syndrome limited scleroderma.
- Exposure to toxins- Certain toxic substances, such as polyvinyl chloride, benzene, silica and trichloroethylene, may trigger scleroderma in patients with a genetic predisposition to the disease.
Complications of CREST Syndrome
The visible signs of CREST syndrome or limited scleroderma are tight, thick skin on the fingers, hands and face, it can change the patients appearance; make everyday tasks, such as opening a jar or shaving, more difficult; and affect the speech. But the most serious complications tend to occur beneath the skin.
- Gastrointestinal problems- Changes in the functioning of esophageal muscles can cause difficulty swallowing and chronic heartburn. When CREST syndrome or limited scleroderma affects the intestine, it can cause constipation, diarrhea, bloating after meals, unintended weight loss and malnutrition.
- Ulcers on fingers and toes- Severe Raynaud’s phenomena can obstruct blood flow to the fingers and toes, causing ulcers that can be difficult to heal. Also, abnormal or narrowed blood vessels combined with severe Raynaud’s phenomena can lead to gangrene of fingers or toes, which might require amputation.
- Lung damage- CREST syndrome or limited scleroderma can cause a variety of problems with the lungs. In some cases, excess collagen collects in the tissue between the lung’s air sacs, making the lung tissue stiffer and less able to work properly. Increased blood pressure in the arteries between the heart and lungs makes the heart work harder and eventually weakens it.
- Heart problems- Scarring of heart tissue can lead to abnormal heart rhythms (arrhythmias) and, in rare cases, to an inflamed heart muscle (myocarditis).
- Kidney problems- Although kidney damage is more common in other forms of scleroderma, it can occur in limited scleroderma. The first indication might be high blood pressure. Restricted blood flow to the kidneys can result in renal crises, which, if untreated, can lead to kidney failure.
- Dental problems- Severe tightening of facial skin can make it difficult to open your mouth wide enough to brush your teeth. Acid reflux (GERD) can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.
- Dry eyes and mouth- CREST syndrome or limited scleroderma can cause very dry eyes and mouth.
Detailed clinical data, elevated levels of antibodies, serology, biomarkers, CENP-B, genetic information, biopsy tissue pathology annotations associated with the CREST syndrome patient’s specimens is provided to a valued customer for drug discover, development and research. The CREST syndrome PBMC, sera (serum) and plasma samples are processed from patients peripheral whole-blood using customized processing protocols provided by the researcher.
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