Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.
What is the outlook (prognosis) for patients with
scleroderma?
A patient's prognosis is optimized with close monitoring of overall
health status and treatment of complications, especially elevated blood
pressure. Recent data indicates that the critical period of
organ risk is generally within the first three years of skin
involvement. This means that patients can be reassured that their
risk of organ-threatening complications is significantly less after
three years of having skin symptoms.
Much more research is needed in all areas of scleroderma
disease, from cause to treatment. Today scleroderma continues to challenge
medical scientists. Researchers are evaluating the
effectiveness of thalidomide (Thalomid) for the treatment of scleroderma. More
sensitive tests to detect early lung disease of scleroderma are also
being evaluated. Psoralen and ultraviolet light therapy (PUVA) is being studied as
a possible treatment for limited scleroderma.
Many researchers are
investigating the roles of various 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 may improve scleroderma. Relaxin normally loosens the ligaments of the pelvis and
ripens the womb for childbirth. How it might work in scleroderma is unclear.
It is clear that our understanding of the effects of the immune system in scleroderma is greatly improving. A vast number of clinical research trials are underway that are evaluating potential treatments for various aspects of the disease to improve the prognosis for the patient.
Scleroderma is a disease of the connective tissue featuring skin thickening, that can involve scarring, blood vessel problems, varying degrees of inflammation, and is associated with an overactive immune system.
Scleroderma is classified into diffuse and limited forms.
CREST syndrome is a limited form of scleroderma.
Patients with scleroderma can have specific antibodies (ANA, anticentromere or antitopoisomerase) in their blood which suggest autoimmunity.
Treatment of scleroderma is directed toward the individual's symptom(s) that is(are) most debilitating.
REFERENCES:
Jimenez, Sergio A., and Patrick M. Cronin. "Scleroderma." eMedicine.com. Jan. 5, 2010. <http://emedicine.medscape.com/article/331864-overview>.
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Co., 2000.
High blood pressure, also known as hypertension, is a repeatedly
elevated blood pressure exceeding 140 over 90 mmHg -- a systolic pressure above
140 with a diastolic pressure above 90. There are two causes of high blood pressure, primary and secondary. Primary high blood pressure is much more common that secondary and its basic causes or underlying defects are not always known. It is known that a diet high in salt increases the risk for high blood pressure, as well as high cholesterol. Genetic factors are also a primary cause. Secondary high blood pressure is generally caused by another condition such as renal hypertension, tumors, and other conditions. Treatment for high blood pressure is generally lifestyle changes and if necessary, diet.
GERD (gastroesophageal reflux disease) is a condition in which the acidified liquid
contents of the stomach backs up into the esophagus. The symptoms of uncomplicated GERD are heartburn,
regurgitation, and nausea. Effective treatment is available for most patients with GERD.
Sjögren's syndrome is an autoimmune disease involving the abnormal production of extra antibodies that attack the glands and connective tissue. Sjögren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Though there is no cure for Sjögren's syndrome, the symptoms may be treated by using lubricating eye ointments, drinking plenty of water, humidifying the air, and using glycerin swabs. Medications are also available to treat dry eye and dry mouth.
Raynaud's phenomenon is characterized by a pale-blue-red sequence of color changes of the digits, most commonly after exposure to cold. Occurring as a result of spasm of blood vessels, the cause is unknown. Symptoms of Raynaud's phenomenon depend on the severity, frequency, and duration of the blood vessel spasm. Treatments include protection of the digits, medications, and avoiding emotional stresses, smoking, cold temperature, and tools that vibrate the hands.
Small intestinal bacterial overgrowth (SIBO) refers to a condition in which abnormally large numbers of bacteria (at least 100,000 bacteria per ml of fluid) are present in the small intestine and the types of bacteria in the small intestine resemble more the bacteria of the colon than the small intestine. There are many conditions associated with small intestinal bacterial overgrowth, to include: diabetes, scleroderma, Crohn's disease, and others. There is a striking similarity between the symptoms of irritable bowel syndrome and SIBO. It has been theorized that SIBO may be responsible for the symptoms of at least some patients with irritable bowel syndrome. Symptoms of SIBO include: excess gas, abdominal bloating, diarrhea, and abdominal pain.
A keloid is a scar that doesn't know when to stop. When the cells keep on reproducing, the result is an overgrown (hypertrophic) scar or a keloid. A keloid looks shiny and is often dome-shaped, ranging in color from slightly pink to red. It feels hard and thick and is always raised above the surrounding skin.
Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes, infections, environmental agents, exposure to ionizing radiation, chronic conditions, and certain medications. Symptoms include shortness of breath, coughing, and diminished exercise tolerance. Treatment options are dependent on the type of pulmonary fibrosis; lung transplant and/or medications are optons.
Dysphagia or difficulty in swallowing, swallowing problems. Dysphagia is due to problems in nerve or muscle control. It is common, for example, after a stroke. Dysphagia compromises nutrition and hydration and may lead to aspiration pneumonia and dehydration.
Pulmonary hypertension is an abnormal elevation of the pressure in the pulmonary circulation caused by the constriction of the blood vessels that supply blood to the lungs. Shortness of breath and dizziness are symptoms of pulmonary hypertension. Treatment involves diuretics, blood thinners, calcium channel blockers, and using supplemental oxygen to increase blood oxygen levels.
Pericarditis is the inflammation of the pericardial sac that surrounds the heart. The causes of pericarditis include injury from heart attack, heart surgery, trauma; viral or fungal infection, HIV, tumors, mixed connective tissue disease, metabolic disease, medication reactions, or idiopathic. Treatment for pericarditis is generally medication, however, sometimes surgery is necessary.
Primary Biliary Cirrhosis is a chronic disease characterized by progressive inflammation and destruction of small bile ducts within the liver. The bile ducts transport bile from the liver to the intestine for the absorption of fat and elimination of waste products. The causes of Primary Biliary Cirrhosis may involve autoimmunity, infection, or genetic predisposition, acting alone or in combination. There are many medications and treatment options available for those with this and other associated diseases of Primary Biliary Cirrhosis.
Interstitial lung disease, is a term to describe a certain lung condition. Causes of interstitial lung disease include lung infection, exposure to toxins in the environment (asbestos for example), medications (chemotherapy), radiation therapy, and chronic autoimmune disorders. Common symptoms of interstitial lung disease include a dry cough and shortness of breath. Diagnosis and treatment depend upon the cause of the condition.
Polymyositis is a disease of the muscle featuring inflammation of the muscle fibers. It results in weakness of the muscles which can be severe and when associated with skin rash, is referred to as dermatomyositis. Although the cause of this disease is unknown, diagnosis includes physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of muscle and nerves, and conformation by a muscle biopsy. Treatment of Polymyositis and Dermatomyositis includes high doses of cortisone-related medications, immune suppression, and physical therapy.
Connective tissue disease is when the body's connective tissues come under attack, possibly becoming injured by inflammation. Inherited connective tissue diseases include Marfan syndrome and Ehlers-Danlos syndrome. Systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymositis, and dermatomyositis are examples of connective tissue diseases that have no known cause.
Connective tissue diseases are disorders featuring abnormalities involving the collagen and elastin.
Connective tissue diseases that are strictly inheritable include Marfan syndrome and Ehlers-Danlos syndrome. The classic immune-related connective tissue diseases include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymyositis, and dermatomyositis. Treatment is often directed at suppressing the inflammation present in the tissues by using anti-inflammatory and immunosuppressive medications.
Preeclampsia is related to increased blood pressure and protein in the mother's urine. Preeclampsia typically begins after the 20th week of pregnancy. When preeclampsia causes seizures, it is termed "eclampsia" and is the second leading cause of maternal death of in the US. Preeclampsia is the leading cause of fetal complications. Risk factors for preeclampsia include high blood pressure, obesity, multiple births, and women with preexisting medical conditions such as diabetes, kidney disease, rheumatoid arthritis, lupus, or scleroderma. Pregnancy planning and lifestyle changes may reduce the risk of preeclampsia during pregnancy.
Bowel or fecal incontinence refers to the loss of voluntary control of stool, or bowel movements. The condition can include partial incontinence, in which a person loses only a small amount of liquid waste, to complete incontinence, in which the entire bowel movement cannot be controlled. Diet changes and elimination of certain medications can help patients to regain bowel control. Treatment involves a combination of medication, biofeedback, and exercise.
Eosinophilic fasciitis is a skin disease that causes thickening and inflammation of the skin and fascia. Symptoms include redness, warmth, and hardening of the skin, as well as occasional tissue and joint pain. Treatment for eosinophilic fasciitis aims to eliminate inflammation through the use of aspirin, NSAIDs, and cortisone. Aggressive forms of eosinophilic fasciitis may require the use of immune suppression medications.
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