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Understanding Scleroderma: Getting the Facts

Contributed by: Nicolette Raya, Pharm.D. Candidate

KU School of Pharmacy

Text Box: What is Scleroderma? Scleroderma is a rare, progressive autoimmune disease that leads to hardening and tightening of the skin and connective tissues – the fibers that provide the framework and support for your body. In some cases, it can also affect blood vessels and internal organs. It is a chronic disease and consists of two types: Localized scleroderma, which mainly affects only the skin and is generally not as destructive or debilitating as systemic scleroderma. Systemic scleroderma is a multi-system disease involving the skin, blood vessels, heart lungs, kidneys, and other major organs. Signs and Symptoms Skin changes include hardening and thickening of skin, ulcers or sores on fingers, loss of hair over affected area, change in skin color, swelling/puffiness in fingers/toes, shiny skin appearance, and disappearance of skin creases. Difficulty making a fist, arthritis, and muscle weakness. Sclerodactyly (hardness of toes/hands). Raynauds’s Phenomenon: telangiectasia (tiny blood vessels seen at the surface of the skin), calcinosis (white calcium lumps under the skin), poor blood flow to extremities, and sensitivity to cold caused by emotional stress. Sjogren’s syndrome (dryness of eyes/mouth). Digestive problems range from poor absorption of nutrients to delayed movement of food as a result of impaired muscular activity in the intes

Text Box: tines. Heart problems can produce scarring of the heart tissue and increases the risk of heart arrhythmias and congestive heart failure. Lung complications include scarring of lung tissue (pulmonary fibrosis) and increased blood pressure in the arteries to the lungs (pulmonary hypertension), all of which decrease lung function. Kidney complications can produce elevated blood pressure in the kidneys and an increased level of protein in the urine. Over time, this can result in renal failure. What Causes it? In scleroderma, the body produces too much collagen which is deposited in body tissues causing thickening and hardening. Collagen is a type of protein that makes up your body’s connective tissues, including your skin. The exact cause of this abnormal collagen production is unknown, but the body’s immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and overproduction of collagen. How is it diagnosed? Physical exam, medical history, blood tests, and tissue samples from the affected skin. Who Gets Scleroderma? The disease usually affects adults between the ages of 30-50, and more women than men. How is Scleroderma Treated? Scleroderma has no known cure. Because

Text Box: there is no treatment to stop the over-production of collagen, medications will only make it easier to live with the disease by treating its symptoms and preventing complications that may affect various organs. In patients with severe systemic scleroderma, pulmonary hypertension is the leading cause of death due to such severe lung dysfunction. How Can I as a Patient Play a Role in the Disease? Stay active. Exercise keeps the body flexible and relieves stiffness. Don’t smoke. Nicotine causes the blood vessels to contract, making Raynaud’s phenomenon worse. Your pharmacist can play a role in smoking cessation. Manage heartburn. This occurs when scleroderma affects the esophagus lining. Protect yourself from the cold. Wear warm gloves for protection during cold temperatures, such as when you reach into a freezer. When outside, cover your face and head and wear layers of warm clothing. Where Can I Find More Information? www.arthritis.org/conditions/DiseaseCenter/scleroderma.asp www.srfcure.org/srf/print/patients/living.htm www.mayoclinic.com/invoke.cfm?id=DS00362