William C. Shiel Jr., MD, FACP, FACR
William C. Shiel Jr., MD, FACP, FACR
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.
Charles Patrick Davis, MD, PhD
Charles Patrick Davis, MD, PhD
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.
In this Article
How is scleroderma diagnosed?
The diagnosis of the scleroderma syndrome is based on the detection of the clinical features of the illness. In addition, nearly all patients with scleroderma have blood tests that suggest autoimmunity and antinuclear antibodies (ANAs) are usually detectable. A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of systemic sclerosis. Anti-Scl 70 antibody (antitopoisomerase I antibody) is most often seen in patients with the diffuse form of systemic sclerosis.
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 stomach and 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?
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Treatment of scleroderma is directed toward the individual features 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 lisinopril, are frequently used.
Some research indicates 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, Lubriderm, and Bag Balm.
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), nicardipine (Cardene), and diltiazem (Cardizem), 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. Drugs that constrict blood vessels, such as pseudoephedrine (Sudafed decongestant) should be avoided. 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. Of note, there is an increased risk of developing lung cancer in persons with scleroderma. This cancer risk is even greater in those who smoke.
Constipation, cramping, and diarrhea are sometimes caused by bacteria that can be treated with tetracycline, erythromycin, ciprofloxin, or metranidazole. Increased fluid intake and fiber intake are good general measures to take to reduce these symptoms. A variety of medications that promote movement of the bowels are available.
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 used to treat pulmonary hypertension. In addition, sildenafil (Revatio) 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). 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, relentless, and can lead to death. Because blood vessel damage is a primary part of the pathology of scleroderma, patients with this disease must not smoke.
Reviewed by Charles Patrick Davis, MD, PhD on 3/6/2013
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