Scleroderma Samples

Bay Biosciences provides high quality, clinical grade fresh frozen bio-specimens, cryogenically preserved sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with scleroderma.

The sera (serum), plasma and PBMC biofluid specimens are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The scleroderma bio-specimens are collected from unique patients diagnosed with scleroderma disease and are provided to a valued pharmaceutical customer for research, diagnostics, discovery and drug development.

Detailed clinical data, scleroderma disease patients history, symptoms, complete blood count (CBC), elevated biomarker levels, genetic and metabolic information, histopathological findings, annotations associated with scleroderma specimens is provided to a valued customer for research, development and drug discovery. The scleroderma disease sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid are processed from patients peripheral whole-blood using customized collection and processing protocols.

Scleroderma Overview

Scleroderma is a chronic autoimmune disease, affecting the skin and other organs of the body, meaning that the body’s immune system is causing inflammation and other abnormalities in the tissue. Scleroderma is also known as progressive systemic sclerosis. The main finding in scleroderma is thickening and tightening of the skin and inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas. Scleroderma is characterized by thickening and tightening of the skin and inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas. Scleroderma affects women more often than men and most commonly occurs between the ages of 30 and 50.

According to the Rheumatology Research Foundation scleroderma is relatively rare, around 75,000 to 100,000 people people in the United States have scleroderma disease. In an an autoimmune disease the body’s immune defenses mistakenly attack the body’s own cells rather than protecting them from outside invaders. There is no cure for scleroderma, but effective treatments for some forms of the disease are available which can ease symptoms and improve quality of life.

There are many different types of scleroderma. In some people, scleroderma affects only the skin. But in many patients, scleroderma also harms structures beyond the skin, such as blood vessels, internal organs and the digestive tract (systemic scleroderma). Signs and symptoms vary, depending on which type of scleroderma you have.

Types of Scleroderma

Following are the two main categories of scleroderma, each category is made up of several conditions.

Localized Scleroderma; often affects only the skin and not major organs.
Systemic Scleroderma: affects the skin and may affect the tissues under it, including blood vessels and major organs, such as the gastrointestinal tract, heart, lungs or kidneys.

There are three types of Localized Scleroderma:

Localized
Generalized
Linear

The skin is typically the only organ impacted in localized scleroderma. However, the tissue injury may extend into the structures underlying the skin, including the subcutaneous tissue, fascia, muscle or bone. Localized scleroderma skin lesions can get better or even go away.

Circumscribed Morphea

With circumscribed morphea (another name for discolored patches of skin), you may have a single oval patch or you may see a few patches of morphea. The patches vary in size and typically have a red border and a thickened pale-yellow center. These lesions can enlarge when active and then flatten and become asymptomatic with treatment. Deep circumscribed morphea extends into the subcutaneous tissues.

Generalized Morphea

Generalized morphea is seen when there are many patches of morphea (greater than four plaques in many anatomical areas (trunk, arms, head or neck). They are seen as thick, tight patches that can blend into each other. Pansclerotic morphea is a severe form of generalized morphea with involvement of most of the body.

Linear Scleroderma

Linear scleroderma is more common in children 10 and younger. These tight, thick bands can appear on the extremities, the back and front of the trunk, the buttocks or the face. They often appear as a single band in one body area and can be seen mixed with patches of morphea. Linear scleroderma can affect the bones and the muscles. When linear scleroderma appears on the arms or legs, it can cause the child’s limb to under develop, causing disability.

Three Types of Systemic Sclerosis (Scleroderma): Limited, Diffuse and Sine

Systemic sclerosis (scleroderma) affects the skin as well as what’s underneath, such as blood vessels, muscles and joints, gastrointestinal (GI) tract, kidneys, lungs and heart.

The skin thickening that accompanies systemic scleroderma can cause tightening so there is a loss of flexibility and ease of movement, especially in the fingers. Facial involvement is common and may be mild or it can reduce facial movements, including decreasing the mouth opening.

Scleroderma may cause chronic joint pain, inflammation and swelling in muscles and joints. Most scleroderma patients also experience Raynaud’s phenomenon, an exaggerated response to ambient temperatures, making one sensitive to cold. Raynaud’s phenomenon causes the skin of the fingers to look white or blue with cold temperature exposure or emotional stress. Skin sores or ulcers can occur due to lack of good blood flow in some cases.

Limited Scleroderma

Limited scleroderma is the most common type of scleroderma. The skin hardening and tightening is limited usually just to the fingers and sometimes the hands, forearms or the face. Internal organ damage is less likely in the limited scleroderma type. In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn, severe Raynaud’s and musculoskeletal pain, and a small subset can develop pulmonary hypertension that can be life-threatening.

A subtype of limited scleroderma is also known as CREST syndrome. CREST is an acronym for its most prominent clinical features:

Calcinosis: calcium deposits under the skin and sometimes in tissues.
Raynaud’s phenomenon: an exaggerated response to ambient temperatures making the skin of the fingers or toes cold, numb or tingling with color changes.
Esophageal dysmotility: which causes heartburn.
Sclerodactyly: the skin on the fingers becomes thick.
Telangiectasias: enlarged blood vessels that appear as red spots on the fingers, face or other parts of the body.

Diffuse Scleroderma

Diffuse scleroderma is a subtype of scleroderma where excess collagen production causes skin thickening over large areas of the body, usually the fingers, hands, arms, anterior trunk, legs and face. There can be significant associated organ damage, including to the gastrointestinal tract, kidneys, lungs and heart. The tightening of the skin is often associated with dryness and itching. Musculoskeletal pain is common. Life-threatening disease occurs when the lung or heart is severely affected. Acute severe systemic high blood pressure can cause kidney damage.

Sine Sclerosis

Sine sclerosis is systemic scleroderma that has features of systemic organ disease, including Raynaud’s phenomenon, but not the skin symptoms. The symptoms and complications of either limited or diffuse scleroderma can occur, but there is no skin thickening.

Signs and Symptoms of Scleroderma

Signs and symptoms of scleroderma’s vary depending on which parts of the body are affected. Following are the common signs and symptoms of scleroderma:

Skin: Almost all scleroderma patients experience a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
Fingers or toes: One of the earliest signs of systemic scleroderma is Raynaud’s disease, which causes the small blood vessels in the fingers and toes to contract in response to cold temperatures or emotional distress. When this happens, the fingers or toes may turn blue or feel painful or numb. Raynaud’s disease also occurs in people who don’t have scleroderma.
Digestive system: Scleroderma can cause a variety of digestive symptoms, depending on which part of the digestive tract is affected. If the esophagus is affected, you might have heartburn or difficulty swallowing. If the intestines are affected, you might have abdominal pain and cramps, bloating, diarrhea or constipation. Some patients who have scleroderma may also have problems absorbing nutrients if their intestinal muscles, food does not move properly through the intestines.
Heart, lungs or kidneys: Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.

Causes of Scleroderma

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up the body’s connective tissues, including skin. Exactly what causes the abnormal collagen production to begin is unknown, but the body’s immune system appears to play a role. Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics and environmental triggers.

In patients with scleroderma, scientists have identified abnormal immune proteins called autoantibodies, which are programmed to attack specific components of body cells. They also have found abnormal accumulations of protective T cells (white blood cells that are part of the immune system) in the skin and elsewhere. Although scientists don’t understand exactly what happens, they believe that the immune system, perhaps involving these autoantibodies or T cells, somehow damages the body’s smallest arteries, called arterioles. These damaged arterioles leak fluid, which causes swelling. They also release chemical factors that stimulate cells called fibroblasts to produce too much collagen, a fibrous protein involved in forming scar tissue.

In the skin, this leads to thickening, hardening and tightness. Elsewhere in the body, the autoimmune attack of scleroderma can damage the digestive tract, the linings of joints, the outside sheaths of tendons, muscles (including the heart muscle), portions of the heart that regulate heart rhythm, the small blood vessels and the kidney.

Scleroderma is a rare disease, affecting about 14 in every 1 million people worldwide. It is most common in women aged 35 to 54. The cause is unknown. For some reason, cells called fibroblasts make too much scar-type tissue in the skin and in organs throughout the body.

A number of theories have been proposed to explain this, including abnormalities in blood vessel function, abnormal proteins and antibodies in the circulation, and abnormal amounts of chemical messengers instructing fibroblasts to become overly active. Because scleroderma is more common in women during the childbearing years, researchers have looked for a pregnancy-related factor to explain why scleroderma develops. One theory suggests that leftover fetal cells can still be circulating in the mother’s bloodstream decades after pregnancy, and may play some role in triggering the autoimmune changes behind scleroderma. Genetic factors and infectious triggers have also been proposed.

Studies have linked scleroderma to exposure to certain chemicals, including vinyl chloride, epoxy resins, aromatic hydrocarbons and ingestion of rapeseed oil adulterated with aniline. Some people who took tryptophan, an amino acid that used to be sold as a dietary supplement, developed a condition similar to scleroderma called eosinophilia myalgia syndrome. Since tryptophan was removed from the market, no further cases of eosinophilia myalgia syndrome have been reported. But the clear link between tryptophan and eosinophilia myalgia syndrome and the scleroderma-like disease associated with contaminated rapeseed oil ingestion raise the possibility that exposure to something in the environment could trigger scleroderma.

Risk Factors of Scleroderma

Anyone can get scleroderma, but it occur much more often in women than in men. Following are some of the various combined factors, which appear to influence the risk of developing scleroderma:

Genetics: People who have certain gene variations appear to be more likely to develop scleroderma. This may explain why a small number of scleroderma cases appear to run in families and why certain types of scleroderma are more common for certain ethnic groups. For example, Choctaw Native Americans are more likely to develop the type of scleroderma that affects internal organs.
Environmental triggers: Research suggests that, in some people, scleroderma symptoms may be triggered by exposure to certain viruses, medications or drugs. Repeated exposure, such as at work, to certain harmful substances or chemicals also may increase the risk of scleroderma.
Immune system problems: Scleroderma is believed to be an autoimmune disease. This means that it occurs in part because the body’s immune system begins to attack the connective tissues. In 15 to 20 percent of cases, a patients who has scleroderma also has symptoms of another autoimmune disease, such as rheumatoid arthritis, Lupus Erythematosus or Sjogren’s syndrome.

Complications of Scleroderma

Scleroderma complications range from mild to severe and can affect the following organs of the body:

Fingertips: The variety of Raynaud’s disease that occurs with systemic scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. In some cases, the tissue on the fingertips may die and require amputation.
Lungs: Scarring of lung tissue can result in reduced lung function, which can impact your ability to breathe, causing shortness of breath and tolerance for exercise. Scleroderma patients may also develop high blood pressure in the arteries to the lungs.
Kidneys: When scleroderma affects the kidneys, you can develop elevated blood pressure and an increased level of protein in the urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
Heart: Scarring of heart tissue increases the risk of abnormal heartbeats and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart. Scleroderma can also raise the pressure on the right side of your heart and cause it to wear out.
Teeth: Severe tightening of facial skin can cause the mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. Patients who have scleroderma often don’t produce normal amounts of saliva, so the risk of dental decay increases even more.
Digestive system: Digestive problems associated with scleroderma can lead to heartburn and difficulty swallowing food. It can also cause bouts of cramps, bloating, constipation or diarrhea.
Sexual function: Men who have scleroderma may experience erectile dysfunction. Scleroderma may also affect the sexual function of women by decreasing sexual lubrication and constricting the vaginal opening.

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