Bay Biosciences provides biopsy tisssue, FFPE blocks, with matched sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with CREST syndrome disease.

Moreover, the samples are collected from unique CREST syndrome patients and are provided to a valued pharmaceutical customer for research, development and drug discovery. 

CREST Syndrome Overview

CREST syndrome is an autoimmune condition that affects the connective tissues. Specifically, the word CREST is an acronym for the condition’s five main features.

Moreover, CREST syndrome impacts various systems in the body, and the symptoms a person experiences can depend on the affected organ. Consequently, doctors use a variety of tests to diagnose CREST syndrome.

Furthermore, due to advancements in treatment, the outlook for those with the condition is improving.

In conclusion, this article explores how CREST syndrome affects the body, along with its causes, diagnosis, and treatments.

What is CREST Syndrome?

CREST syndrome, also known as Limited Scleroderma (subtype of Scleroderma) or limited cutaneous form of Systemic Sclerosis (LcSSc), is, in fact, a multi-system connective tissue disorder.

Specifically, CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas; furthermore, its name is an acronym for the cardinal clinical features of the syndrome.

In addition, the acronym “CREST” refers to the five main features: Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

Notably, the skin changes (hardened skin) associated with limited scleroderma typically occur only in the lower arms and legs, specifically below the elbows and knees, and sometimes affect the face and neck. Moreover, limited scleroderma can also affect the digestive tract, heart, lungs, or kidneys.

Although the problems caused by limited scleroderma may be minor, sometimes, however, the disease affects the lungs or heart, with potentially serious results. Ultimately, CREST syndrome or limited scleroderma has no known cure. Consequently, 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. The symptoms of CREST syndrome vary depending on the affected organs.

Aside from the five clinical elements mentioned above, common symptoms include:

  • First, heart problems
  • Next, swelling and stiffness in the joints
  • In addition, muscle weakness, pain, or stiffness
  • Moreover, inflammation in areas such as the hands or face
  • Additionally, kidney problems
  • Lastly, scarring of the lungs that affects breathing (pulmonary fibrosis)

Causes of CREST Syndrome

Scientists are unsure of the exact cause of scleroderma and CREST syndrome. Nevertheless, it appears to be an autoimmune condition. In other words, this means that the immune system attacks the body’s connective tissue.

Several factors likely interact to cause CREST syndrome and scleroderma. For instance, 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.

Moreover, in CEST syndrome, the immune system appears to stimulate the production of too much collagen. Furthermore, which a key component of connective tissue. Consequently, this overproduction of collagen builds up in the skin and internal organs so that they don’t function normally.

Ultimately, CREST syndrome does not appear to pass from parent to child.

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.

Diagnosis of CREST Syndrome

Doctors diagnoseTrusted Source CREST syndrome by considering a person’s medical history and physical exams. In addition, various tests, either routine or specialized, can also be beneficial.

Specifically, the process can include:

  • First, blood tests to check for signs of inflammation and autoimmunity
  • Second, checking the skin for swelling, thickening, and tightening
  • Moreover, electromyography (EMG) and nerve conduction studies
  • Furthermore, creatinine kinase tests to measure muscle involvement
  • In addition, imaging tests such as MRI, CT, or X-ray, to assess for any atypical growths
  • Likewise, pulmonary function Tests (PFT) to assess breathing
  • Finally, serum creatinine tests to measure kidney function

Treatments of CREST Syndrome

Moving on to the next topic, TREATMENT. Depending on their symptoms, a person may require a combination of treatments and/or other management strategies.

Ultimately, the primary goals of treatment are to:

  • Improve quality of life
  • Limit the progression of the disease
  • Reduce inflammation

Moreover, treatment options may include:

  • Anti-inflammatory drugs: Aspirin, indomethacin (Indocin), and naproxen (Naprosyn) can reduce inflammation.
  • D-penicillamine and colchicine: These medications can help control the hardening of the skin and internal organs.
  • Immunosuppressants: Steroids may slow disease progression and limit flare-ups.
  • Laxatives or heartburn medication: These medications can reduce digestive symptoms.
  • Vasodilators: These medications ease Raynaud’s symptoms and prevent lung and kidney damage.

Therefore, people should work with their doctor for individualized treatment plans to control their symptoms.

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.

Dental Problems

  • 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

  • Dry eyes and mouth- CREST syndrome or limited scleroderma can cause very dry eyes and mouth.

Gastrointestinal Problems

  • 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.

Heart Problems

  • 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

  • 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.

Lung Damage

  • 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.

Ulcers on Fingers and Toes

  • 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.

Biospecimens

biospecimens

Bay Biosciences is a global leader in providing researchers with high quality, clinical grade, fully characterized human tissue samples, bio-specimens, and human bio-fluid collections.

Moreover, human biospecimens are available including tumor tissue, serum, plasma and PBMC samples from most other therapeutic areas.

Furthermore, Bay Biosciences maintains and manages its own biorepository, the human tissue bank (biobank) consisting of thousands of diseased samples (specimens) and likewise normal healthy donors for controls. Additionally, available in all formats and types.

In fact, our biobank procures and stores fully consented, de-identified and institutional review boards (IRB) approved human tissue samples, human biofluids such as serum samples, plasma samples from various diseases and matched controls.

Also, all our human tissue collections, human biospecimens and human biofluids are provided with detailed, samples associated patient’s clinical data.

In fact, this critical patient’s clinical data includes information relating to their past and current disease, treatment history, lifestyle choices, biomarkers, and genetic information.

Additionally, researchers find the patient’s data associated with the human biospecimens extremely valuable and use it to help identify new effective treatments (drug discovery & development) in oncology, as well as in other therapeutic areas and diseases.

Bay Biosciences banks wide variety of human tissue samples and human biological samples, including fresh frozen human biospecimens cryogenically preserved at – 80°C.

For example fresh frozen tissue samples, tumor tissue samples, formalin-fixed paraffin-embedded (FFPE), tissue slides, with matching human bio-fluids, whole blood and blood-derived products such as human serum, human plasma and human PBMCs.

Bay Biosciences is a global leader in collecting and providing human tissue samples according to the specified requirements and customized, tailor-made collection protocols.

Please contact us anytime to discuss your special research projects and customized human tissue sample requirements.

Types of Biospecimens

Bay Biosciences provides human tissue samples (human specimens) and human biofluids from diseased and normal healthy donors which includes:

Moreover, we can also procure most human biospecimens and human biofluids, special collections and requests for human samples that are difficult to find. All our human tissue samples and human biofluids are procured through IRB-approved clinical protocols and procedures.

In addition to the standard processing protocols, Bay Biosciences can also provide human biofluids such as  human plasma, human serum, and human PBMCs bio-fluid samples using custom processing protocols; you buy donor-specific collections in higher volumes and specified sample aliquots from us.

Bay Biosciences also provides human biospecimens from normal healthy donors; volunteers, for controls and clinical research, Contact us Now.