SclerodermaThe definition of Scleroderma
Scleroderma is an autoimmune disease of connective tissue. Autoimmune diseases are diseases that occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs. This leads to thickness and firmness of involved areas. Scleroderma, when he scattered or widely spread throughout the body, also referred to as systemic sclerosis.
The cause of scleroderma is unknown. Researchers have found some evidence that genes are important factors, but the environment also seems to play a role. The result is activation of the immune system, causing injury to the tissues that results in injury similar to scar tissue formation. The fact that genes seem to cause a tendency to develop scleroderma at least mean that inheritance plays a part role. It is not unusual to find other autoimmune diseases in families of scleroderma patients. Some evidence for a possible role played by genes in the lead on the development of scleroderma comes from the study of Choctaw Native Americans who are a group with a reported incidence of the disease the most high. The disease is more common in women than in men.
Classification of Scleroderma
Scleroderma can be classified in terms of degree and location of involved skin. Therefore, scleroderma has been categorized into two major groups, diffuse (spread) and limited (limited).
Diffuse form of scleroderma (systemic sclerosis) involves symmetric thickening of the skin of the foot-kai and the hands, face and torso (chest, back, abdomen, or pelvis-pelvic) pliers can be quickly developed / evolved to hardening after an early inflammatory phase. Organ disease can occur early and are serious. Organs affected include the esophagus (esophageal), intestines, lungs with scarring (fibrosis), heart, and kidneys. High blood pressure can be a troublesome side effect.
Limited form of scleroderma tends to be limited to the skin of the fingers and face. Changes in skin and other features of the disease tends to occur more slowly than the diffuse form. Because a characteristic clinical pattern can occur in patients with limited forms of scleroderma, this form has taken another name which is composed of the beginning of the first letters of the common components. Thus, this form is also called the CREST variant of scleroderma. This name indicates the following characteristics:
C. .. calcinosis refers to the formation of tiny deposits of calcium in the skin. This is seen as whitish areas are hard on the superficial skin, generally covering the elbow-elbow, knees, or fingers. Solid deposits can be tender, can menkadi infected, and can fall off spontaneously or require surgical removal. This is the least common of the features of the CREST scleroderma variant.
R. .. Raynaud's phenomenon refers to the seizure of vessels are small arteries that mensupali blood to the fingers, toes, nose, tongue, or ears. These areas became blue, white, then red after exposure to extremes of cold, or even sometimes with extremes of heat or emotional disturbance.
E. .. Esophagus disease in scleroderma is characterized by the functioning of the muscles of the lower two-thirds of the esophagus that bad. This can lead to esophageal suau abnormally wide which allows stomach acid to flow back into the esophagus to cause heartburn (a burning sensation dihulu liver), inflammation, and scarring potential. This can eventually lead to difficulty in passing food from the mouth through the esophagus into the stomach. The symptoms of heartburn are treated aggressively in patients with scleroderma in order to prevent injury to the esophagus.
S. .. sclerodactyly refers to the thickening and tightening skin in place of fingers or toes. This can give them a vision of "shimmering" and a little swollen. Tightening can cause severe restriction of movement of the fingers and toes. These skin changes generally continue more slowly than that of patients with diffuse form of scleroderma.
T. .. Telangiectasias are areas of small red, often on the face, hands and mouth behind the lips. These areas blanch when they are pressed on top and represent a dilated capillaries.
Patients can have variations from CREST, for example, CRST, REST, ST, and so on. Patients may also have the disease "overlap" with characteristics of both CREST and the diffuse form of scleroderma. Some patients have overlap-overlap of scleroderma and connective tissue diseases, such as, systemic lupus erythematosus, and polymyositis. When the characteristics of scleroderma are present along with the characteristics of polymyositis and systemic lupus erythematosus, a condition referred to as mixed connective tissue disease or mixed connective tissue disease (MCTD).
Finally, these changes can be very localized scleroderma skin. Morphea scleroderma skin is localized in a field area of the skin becomes hardened and slightly pigmented. Sometimes morphea can cause various injuries to the skin. Morphea not associated with disease elsewhere in the body. Linear scleroderma is scleroderma is typically located at a distance, often presented as an area of hardened skin down the leg of a child. Linear scleroderma in children can slow the growth of bone from the affected limb. Sometimes linear scleroderma associated with an area of "satellite" of a small area of skin of localized scleroderma, such as the stomach.
Symptoms of Scleroderma
The symptoms of scleroderma depends on the type of scleroderma present and extent of involvement of external and internal to the individuals affected. Because scleroderma can affect the skin, esophagus, blood vessels, kidneys, lungs, blood pressure and intestines, the symptoms it causes can involve many areas of the body.
Scleroderma affects the skin to cause signs of inflammation are in place (local) or widespread (redness, swelling, tenderness, itching, and pain) that can lead to a tightening or hardening of the skin. These skin changes can be widespread, but is most common for them to affect the fingers, feet, face and neck. This can lead to a reduced restriction of movement of the fingers, toes, and jaw. Small areas of calcification or calcification (calcinosis), while not common, can sometimes be noted as a hard nodules at the ends of the bracket arms or fingers.
Scleroderma affecting the esophagus (esophageal) lead to heartburn. This is directly as a result of stomach acid flows back up into the esophagus. Sometimes this can lead to scarring of the esophagus with difficulty swallowing and / or localized pain in the center of the chest.
Blood vessels that can be affected including the arterioles, small arterioles of the ends of fingers, toes, and elsewhere. These vessels may have a tendency to kink when the areas exposed to cold, leading to the bluish, pallor, and redness of the fingers, toes, and sometimes the nose or ears are involved. These color changes are referred to as Raynaud's phenomenon. Raynaud's phenomenon can cause an insufficient oxygen supply to the ends of the fingers or toes are involved, causing small ulcers or skin is blackened (dead). Sometimes Raynaud's phenomenon is also associated with a tingling sensation (tingling). Other blood vessels that DAPT involved in scleroderma is small capillaries of the face, lips, mouth, or fingers. Capillaries are dilated to form small red spots that blanch, called telangiectasias.
Elevated blood pressure is potentially serious and can lead to kidney damage. Symptoms include headache, fatigue, and in severe cases, stroke.
Inflammation of the lungs in scleroderma can cause scarring, resulting in shortness of breath, especially with physical exertion. Rising pressure in the arteries of the lungs (pulmonary hypertension) can also cause shortness of breath and difficulty breathing memdapatkan a considerable activity.
Scleroderma affecting the large intestine (colon) most often causes constipation but can also lead to cramps and diarrhea. When this is the heavy / severe, can result in barriers defecation completely.
Diagnosing SclerodermaThe diagnosis of scleroderma syndrome is based on the discovery of the clinical characteristics of diseases. Almost all patients with scleroderma have blood tests which suggest autoimmunity, antinuclear antibodies (ANAs). A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of scleroderma. Anti-SCL 70 antibody (antitopoisomerase I antibody) is most Often seen in Patients with the diffuse form of scleroderma.
Other tests used to evaluate the presence or extent of any internal disease (in). This may include tests of upper and lower digestive tract to evaluate the intestines, chest x-rays, pulmonary function testing, and CAT scanning to examine the lungs, ECG and echocardiograms, and sometimes cardiac catheterization to evaluate the pressure in the arteries from the heart and lungs.Treating Scleroderma
Treatment of scleroderma is directed toward the individual characteristics that affect areas of the body are different.
Aggressive treatment of the elevation-elevation in blood pressure has become very important in preventing kidney failure. Blood pressure medications, such as captopril, are often used.
Recent data indicate that colchicine may be useful in reducing inflammation and tenderness that periodically accompanies the calcinosis nodules on the skin. Itching skin can be freed with lotion-lotion (emollients) such as Eucerin and Lubriderm.
Raynaud's phenomenon may require only mild heating and hand protection. Low-dose aspirin is often added to prevent small blood clots in the fingers, especially in patients with a history of ulceration, ulceration of the fingertips. Raynaud's phenomenon that is can be helped by medications that open the arteries, such as nifedipine (Procardia, Adalat) and nicardipine (Cardene), or with topical (topical) nitroglycerin applied to the fingers / legs are most affected (the most effective on the sides of the fingers / legs where the arteries are). Buffer is applied gentle finger can protect tender tissues. A group of medications that are typically used for depression, called serotonin reuptake inhibitors, like fluoxetine (Prozac), can sometimes improve the circulation of the fingers / legs are affected. Raynaud's phenomenon severe / severe to require surgical procedures, such as to interrupt the nerve-syraf of fingers which stimulates constriction of blood vessels (digital sympathectomy). Ulceration, ulceration of the fingers may require topical antibiotics (topical) or oral (mouth).
Irritation of the esophagus and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids may also be useful. Raising the head of the bed can reduce backflow of acid into the esophagus causing inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.
Constipation, cramps, and diarrhea is sometimes caused by bacteria that can be treated with tetracycline or erythromycin. These studies have recently shown that erythromycin can also be used. Fluid intake and fiber enhanced measures are generally good.
Dry, irritated skin and itching can be helped with emollients such as Lubriderm, Eucerin, or Bagbalm.
Telangiectasias, as on the face, can be treated with local laser therapy. Exposure to sunlight should be minimized because it can worsen telangiectasias.
Approximately 10% of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension). Abnormally elevated blood pressure of the arteries that supply the lungs is often treated with calcium antagonist drugs, such as nifedipine, and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by intravenous infusion of prostacyclin (Iloprost) are continuous. A new drug is taken, bosentan (Tracleer), is now available to treat pulmonary hypertension severe / severe.
In addition, drugs used to suppress an active immune system that seems excessive spontaneously cause disease in the affected organs. Drugs that are used for this purpose include penicillamine, azathioprine, and methotrexate. Recent research has found that low-dose penicillamine is as effective as high doses of penicillamine previously used, with less toxicity. Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone. Optimal treatment of scleroderma lung disease is an area of active research. Stem cell transplantation (stem-cell transplantation) is being explored as a possible option.
No cure has been found that is universally effective for all patients with scleroderma. On an individual patient, the illness may be mild and require no treatments. In some, the disease is destroyed and without mercy.
Prognosis for Patients-Patients With Scleroderma
The prognosis of a patient is optimized with observations (monitor) the stringent requirements of the overall health status and treatment of complications, especially blood pressure is rising. Recent data indicate that the critical period of risk organs is generally within the first three years of skin involvement. This means that patients can be reassured her that the risk of complications that threaten their organs were significantly less after three years has symptoms kuliut.
More research is needed in all areas of scleroderma disease, from causes to treatments. Today scleroderma continues astonishing medical scientists. Researchers are evaluating the effectiveness of thalidomide for the treatment of scleroderma. These tests are more sensitive for detecting early lung disease of scleroderma are also being evaluated. Psoralen and ultraviolet light therapy (PUVA) is being studied as a possible treatment for scleroderma is limited.
Many researchers are investigating the roles of diverse cell messengers, called cytokines, in causing scleroderma. Researchers are also currently studying a hormone of pregnancy, called relaxin, for the treatment of scleroderma. Preliminary results suggest that it might ameliorate scleroderma. Relaxin normally loosen the ligaments of the pelvis and uterus to the birth of a child mature. How it might work in scleroderma is not clear.
Symptoms of Scleroderma
The symptoms of scleroderma depends on the type of scleroderma present and extent of involvement of external and internal to the individuals affected. Because scleroderma can affect the skin, esophagus, blood vessels, kidneys, lungs, blood pressure and intestines, the symptoms it causes can involve many areas of the body.
Scleroderma affects the skin to cause signs of inflammation are in place (local) or widespread (redness, swelling, tenderness, itching, and pain) that can lead to a tightening or hardening of the skin. These skin changes can be widespread, but is most common for them to affect the fingers, feet, face and neck. This can lead to a reduced restriction of movement of the fingers, toes, and jaw. Small areas of calcification or calcification (calcinosis), while not common, can sometimes be noted as a hard nodules at the ends of the bracket arms or fingers.
Scleroderma affecting the esophagus (esophageal) lead to heartburn. This is directly as a result of stomach acid flows back up into the esophagus. Sometimes this can lead to scarring of the esophagus with difficulty swallowing and / or localized pain in the center of the chest.
Elevated blood pressure is potentially serious and can lead to kidney damage. Symptoms include headache, fatigue, and in severe cases, stroke.
Inflammation of the lungs in scleroderma can cause scarring, resulting in shortness of breath, especially with physical exertion. Rising pressure in the arteries of the lungs (pulmonary hypertension) can also cause shortness of breath and difficulty breathing memdapatkan a considerable activity.
Scleroderma affecting the large intestine (colon) most often causes constipation but can also lead to cramps and diarrhea. When this is the heavy / severe, can result in barriers defecation completely.
Other tests used to evaluate the presence or extent of any internal disease (in). This may include tests of upper and lower digestive tract to evaluate the intestines, chest x-rays, pulmonary function testing, and CAT scanning to examine the lungs, ECG and echocardiograms, and sometimes cardiac catheterization to evaluate the pressure in the arteries from the heart and lungs.Treating Scleroderma
Treatment of scleroderma is directed toward the individual characteristics that affect areas of the body are different.
Aggressive treatment of the elevation-elevation in blood pressure has become very important in preventing kidney failure. Blood pressure medications, such as captopril, are often used.
Recent data indicate that colchicine may be useful in reducing inflammation and tenderness that periodically accompanies the calcinosis nodules on the skin. Itching skin can be freed with lotion-lotion (emollients) such as Eucerin and Lubriderm.
Raynaud's phenomenon may require only mild heating and hand protection. Low-dose aspirin is often added to prevent small blood clots in the fingers, especially in patients with a history of ulceration, ulceration of the fingertips. Raynaud's phenomenon that is can be helped by medications that open the arteries, such as nifedipine (Procardia, Adalat) and nicardipine (Cardene), or with topical (topical) nitroglycerin applied to the fingers / legs are most affected (the most effective on the sides of the fingers / legs where the arteries are). Buffer is applied gentle finger can protect tender tissues. A group of medications that are typically used for depression, called serotonin reuptake inhibitors, like fluoxetine (Prozac), can sometimes improve the circulation of the fingers / legs are affected. Raynaud's phenomenon severe / severe to require surgical procedures, such as to interrupt the nerve-syraf of fingers which stimulates constriction of blood vessels (digital sympathectomy). Ulceration, ulceration of the fingers may require topical antibiotics (topical) or oral (mouth).
Irritation of the esophagus and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids may also be useful. Raising the head of the bed can reduce backflow of acid into the esophagus causing inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.
Constipation, cramps, and diarrhea is sometimes caused by bacteria that can be treated with tetracycline or erythromycin. These studies have recently shown that erythromycin can also be used. Fluid intake and fiber enhanced measures are generally good.
Dry, irritated skin and itching can be helped with emollients such as Lubriderm, Eucerin, or Bagbalm.
Telangiectasias, as on the face, can be treated with local laser therapy. Exposure to sunlight should be minimized because it can worsen telangiectasias.
Approximately 10% of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension). Abnormally elevated blood pressure of the arteries that supply the lungs is often treated with calcium antagonist drugs, such as nifedipine, and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by intravenous infusion of prostacyclin (Iloprost) are continuous. A new drug is taken, bosentan (Tracleer), is now available to treat pulmonary hypertension severe / severe.
In addition, drugs used to suppress an active immune system that seems excessive spontaneously cause disease in the affected organs. Drugs that are used for this purpose include penicillamine, azathioprine, and methotrexate. Recent research has found that low-dose penicillamine is as effective as high doses of penicillamine previously used, with less toxicity. Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone. Optimal treatment of scleroderma lung disease is an area of active research. Stem cell transplantation (stem-cell transplantation) is being explored as a possible option.
No cure has been found that is universally effective for all patients with scleroderma. On an individual patient, the illness may be mild and require no treatments. In some, the disease is destroyed and without mercy.
The prognosis of a patient is optimized with observations (monitor) the stringent requirements of the overall health status and treatment of complications, especially blood pressure is rising. Recent data indicate that the critical period of risk organs is generally within the first three years of skin involvement. This means that patients can be reassured her that the risk of complications that threaten their organs were significantly less after three years has symptoms kuliut.
More research is needed in all areas of scleroderma disease, from causes to treatments. Today scleroderma continues astonishing medical scientists. Researchers are evaluating the effectiveness of thalidomide for the treatment of scleroderma. These tests are more sensitive for detecting early lung disease of scleroderma are also being evaluated. Psoralen and ultraviolet light therapy (PUVA) is being studied as a possible treatment for scleroderma is limited.
Many researchers are investigating the roles of diverse cell messengers, called cytokines, in causing scleroderma. Researchers are also currently studying a hormone of pregnancy, called relaxin, for the treatment of scleroderma. Preliminary results suggest that it might ameliorate scleroderma. Relaxin normally loosen the ligaments of the pelvis and uterus to the birth of a child mature. How it might work in scleroderma is not clear.
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