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.
The diagnosis of the scleroderma syndrome is based on the finding of the clinical features of the illnesses. In addition, nearly all patients with scleroderma have blood tests that suggest autoimmunity, because antinuclear antibodies (ANAs) are usually detectable. 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 are used to evaluate the presence or extent of any internal disease. These
may include upper and lower gastrointestinal tests to evaluate the
bowels, chest X-rays, lung-function testing
(pulmonary function test), and CAT scanning to examine the lungs,
EKG and echocardiograms, and sometimes heart catheterization to evaluate the pressure in the arteries of the heart and lungs.
What is the treatment for scleroderma?
Treatment of scleroderma is directed toward the individual feature(s) affecting different areas
of the body.
Aggressive treatments of elevations in blood pressure have been extremely important in preventing kidney failure. Blood-pressure medications, particularly the angiotensin converting enzyme (ACE) inhibitor class of drugs, such as captopril (Capoten), are
frequently used.
Recent data indicate that colchicine can be
helpful in decreasing the inflammation and tenderness that periodically
accompanies the calcinosis nodules in the skin. Skin itching can be
relieved with lotions (emollients) such as Eucerin and Lubriderm.
Mild Raynaud's phenomenon may require only hand warming and
protection. Low-dose aspirin is often added to prevent tiny blood clots in the fingers, especially in patients with a history of
fingertip ulcerations. Moderate Raynaud's phenomenon can be helped by
medications that open up the arteries, such as
nifedipine (Procardia, Adalat) and nicardipine (Cardene), or with
topical nitroglycerin applied to the most affected digit (most effective on the sides of the digit where the arteries are). Gently
applied finger splinting can protect tender tissues. (It is important to not constrict the tiny arteries on the sides of the fingers when protecting them with splints, braces, or band-aid materials.) A class of medications that is typically used for depression, called serotonin reuptake inhibitors, such as fluoxetine (Prozac), can sometimes improve the circulation of the affected digit. Severe Raynaud's
phenomenon can require surgical procedures, such as those to
interrupt the nerves of the finger that stimulate constriction of the
blood vessels (digital sympathectomy). Ulcerations of the fingers can
require topical or oral antibiotics.
Esophagus irritation and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or
lansoprazole (Prevacid). Antacids can also be helpful. Elevating the head of the bed can reduce
the back-flow of acid into the esophagus that causes inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.
Constipation, cramping, and diarrhea is sometimes caused by bacteria that can be
treated with tetracycline or erythromycin.
Studies have shown that erythromycin could also be used. Increased fluid intake and fiber intake are good general measures.
Irritated, itchy dry skin can be helped by emollients such as Lubriderm, Eucerin, Bag
Balm histamine 2 blockers, or trazodone (Desyrel).
Telangiectasias, such as those on the face,
can be treated with local laser therapy. Sun exposure should be minimized as 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
supplying the lungs is often treated with calcium antagonist medications, such as nifedipine
(Procardia), and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension
can be helped by continuous intravenous infusion or inhalation of prostacyclin (Iloprost).
Taken by mouth, bosentan (Tracleer), is now also available to treat pulmonary hypertension. In addition, sildenafil (Viagra) and tadalafil (Cialis) have been FDA approved to treat pulmonary hypertension.
Additionally, medications are used to suppress the
overly active immune system that seems to be spontaneously causing the
disease in organs. Medications used for this purpose include
penicillamine, azathioprine (Imuran, Azasan), and methotrexate (Rheumatrex, Trexall). Recent research
has found that low-dose penicillamine (Depen, Cuprimine) (125 mg every other day) is as
effective as previously used high doses of penicillamine, with less toxicity. Serious
inflammation of the lungs (alveolitis) can
require immune suppression with cyclophosphamide (Cytoxan) along with
prednisone (Deltasone, Liquid Pred). The optimal treatment of scleroderma lung disease is an area of active research. Stem-cell transplantation is being explored as a possible option.
No medication has been found to be universally effective for all patients
with scleroderma. In an individual patient, the illness may be mild and not require
treatments. In some, the disease is ravaging and relentless.
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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