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Article #293: Scleroderma - Definition, Causes, Symptoms and Treatment

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Scleroderma also known as systemic Scleroderma affects approximately 300,000
sclerosis. Scleroderma is an autoimmune, people in the United States. Scleroderma
connective tissue disease. Scleroderma affecting the esophagus leads to
causes inflammation and thickening of the heartburn. This is directly a result of
skin. Genes may play in leading to the stomach acid backflowing up into the
development of scleroderma. Scleroderma esophagus. Sometimes this can lead to
is a diffuse connective tissue disease scarring of the esophagus with difficulty
characterized by changes in the skin, swallowing and/or localized pain in the
blood vessels, skeletal muscles, and central chest. It is four times as
internal organs. A connective tissue common in women than in men. Incidence
disease is one that affects tissues such rates are estimated at 2-20 per million
as skin, tendons, and cartilage. In some per year in the United States.
cases, scleroderma also affects the blood Scleroderma affecting the large bowel
vessels and internal organs. Scleroderma (colon) most often causes constipation,
is one of a group of arthritic conditions but can also lead to cramping and
called connective tissue disorders. In diarrhea. When this is severe, it
these disorders, a person's antibodies complete stool blockage (fecal impaction)
are directed against his or her own can result. Inflammation of the lungs in
tissues. Scleroderma can be classified in scleroderma can cause scarring, resulting
terms of the degree and location of the in shortness of breath, especially with
skin involvement. Accordingly, physical exertion. Elevated pressure in
scleroderma has been categorized into two the arteries to the lungs (pulmonary
major groups, diffuse and limited. hypertension) can also cause shortness of
Diffuse form of scleroderma is involves breath and difficulty getting an adequate
symmetric thickening of skin of the breath with activity. Elevated blood
extremities, face, trunk (chest, back, pressure is potentially serious and can
abdomen, or flanks) which can rapidly lead to kidney damage. Symptoms include
progress to hardening after an early headache, fatigue, and in severe cases,
inflammatory phase.Organ disease can stroke.
occur early on and be serious. Organs Scleroderma can affect many different
affected include the esophagus, bowels, organs and organ systems. Exposure to
lungs with scarring (fibrosis), heart, industrial solvents or an environmental
and kidneys. agent may play a role in predisposing to
Limited scleroderma typically has gradual scleroderma. Medications that help with
onset and is restricted to certain areas blood circulation such as,
of the skin. Raynaud's phenomenon is due angiotensin-converting enzyme (ACE)
to vasoconstriction of the small arteries inhibitors and low-dose enteric-coated
of exposed peripheries - particularly the aspirin. Hydroxychloroquine (Plaquenil)
hands and feet - in the cold. The skin drug has relatively few side effects, and
changes and other features of disease it's also effective for the arthritis
tend to occur more slowly than in the that can be associated with scleroderma.
diffuse form. Because a characteristic Apart from hydroxychloroquine's apparent
clinical pattern can occur in patients ability to affect the way immune cells
with the limited form of scleroderma, work, scientists don't completely
this form has taken another name which is understand how it helps tame the disease
composed of the first initials of the process. Cyclophosphamide (Cytoxan) is
common components. Telangiectasia a sometimes used to treat pulmonary
condition caused by the swelling of tiny fibrosis. People with scleroderma-related
blood vessels, in which small red spots lung disease found cyclophosphamide
appear on the hands and face. While not modestly improved lung function and
painful, these red spots can create quality of life. Bosentan (Tracleer) is
cosmetic problems. Genetic predisposition an oral medication that has been approved
appears to be limited: genetic for pulmonary hypertension in people with
concordance is small; still, there often scleroderma. Esophagus irritation and
is a familial predisposition for heartburn can be relieved with omeprazole
autoimmune disease. Polymorphisms in (Prilosec), esomeprazole (Nexium), or
COL1A and TGF-ß1 may influence severity lansoprazole (Prevacid). Antacids can
and development of the disease. There is also be helpful. Elevating the head of
limited evidence implicating the bed can reduce backflow of acid into
cytomegalovirus (CMV) as the original the esophagus that causes inflammation
epitope of the immune reaction, and and heartburn. Avoiding caffeine and
organic solvents and other chemical cigarette smoking also helps.
agents have been linked with scleroderma.






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