NUR 101- Ms. Lee Extra Credit The Facts About Scleroderma Scleroderma is a chronic autoimmune disease that was first described in the 18 th century. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposits of collagen. There are two types of scleroderma. Localized scleroderma affects the skin in limited areas and the musculoskeletal system.
Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys. It can cause arthritis, slow contractions in the gastrointestinal tract, muscle inflammation, dry eyes and dry mouth. Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Raynaud's phenomenon, which causes the fingers or toes to turn white or blue and may be painful (web). Localized Scleroderma is more common in children. It is usually found in only a few places on the skin or in the muscles and rarely, if ever, does localized scleroderma develop into the systemic form of the disease. Systemic Sclerosis may affect the connective tissue in many parts of the body, e.
g. the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart and other internal organs. It may also affect blood vessels, muscles and joints and is life threatening Scleroderma can range from a minor skin lesion to a debilitating and fatal disease. (web). In most cases, the cause of scleroderma is unknown. However, in a small minority of cases, scleroderma or scleroderma-like illnesses are associated with exposure to certain toxins or as a complication of bone marrow transplants.
Scleroderma is not contagious and is rarely inherited. Systemic sclerosis is associated with over-activation of the immune system, which normally functions to protect the body against cancers and invading infections. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue (web). Skin changes include hardening and thickening, ulcers or sores on fingers, loss of hair over affected area, change in skin color, swelling / puffiness in fingers / toes and the skin appears shiny.
Usually skin creases disappear, there becomes difficulty in making a fist, and Sclerodactyly (hardness of toes / hands ) occurs. Raynaud's Phenomenon may include poor blood flow to extremities, sensitivity to cold caused by emotional stress, telangiectasis (tiny blood vessels seen at the surface of the skin), calcinosis (white calcium lumps under the skin), arthritis and muscle weakness. Sjogren's syndrome includes dryness of eyes and the mouth and digestive problems and heart, lung, and kidney problems (web). Although scleroderma strikes every age, sex and ethnic background, four times more females, between the ages of 25 and 55, are afflicted with scleroderma than males. The frequency increases with age in both sexes. Scleroderma is far more prevalent than muscular dystrophy or multiple sclerosis (web).
Scleroderma affects women more than men and adults more than children. 10-20 new cases are diagnosed per million people each year. Five-year survival rate is 80 - 85 percent. Lung, heart and kidney damage are the most frequent causes of severe disability and death. Many people have decreased hand function because of joint disfigurement or finger ulcers (web). Although the cause is unknown, it is known that the body produces too much of a protein called collagen.
Excess collagen is deposited causing thickening and hardening (web). Diagnosis of scleroderma is based on clinical history and physical findings. Diagnosis may be delayed in those without significant skin thickening. The diagnostic process may require consultations with rheumatologist (arthritis specialists) and dermatologists (skin specialists) as well as blood studies and a variety of specialized tests depending upon which organs are affected. The treatment program varies depending on the type and severity of the symptoms. Diagnosis may be very difficult, particularly in the early stages, because many of the symptoms are common to, or may overlap with those of other diseases, especially other connective tissue diseases such as rheumatoid arthritis, lupus, and poly myositis (web).
Early diagnosis is important in allowing initiation of treatments designed to prevent long-term complications. Medications are commonly used. Surgery may be required for some conditions. Research is investigating bone marrow transplants for persons with scleroderma (web). Laboratory, X-ray and pulmonary function tests determine the extent and severity of internal organ involvement. Physical exam, medical history and lab tests which may include biopsy and blood tests.
There is no known cure for scleroderma. No treatment has been scientifically proven to alter the overall course of the disease, although d-penicillamine is commonly used for this purpose and may be of some value. There are a number of effective organ-specific treatments for scleroderma. Raynaud's phenomenon may be helped by calcium channel blockers.
Declining renal function and hypertension are often treated with drugs. Esophageal damage from reflux of stomach contents can be treated with acid-reducing drugs. Antibiotics, special diets and medication can improve absorption of nutrients in people who have abnormalities of their intestines. Musculoskeletal pain may respond to nonsteroidal anti-inflammatory agents. Lung inflammation may be treated with cyclophosphamide (web). Researchers say a common antibiotic that has been in used for more than 20 years has proven effective against a potentially fatal skin disease from which thousands of Americans suffer.
People in the early stages of scleroderma can get total relief from the debilitating autoimmune disease by taking the antibiotic minocycline twice a day, according to the findings of a year-long pilot study. "The results are highly significant," said study leader Dr. David Trentham, a rheumatologist at Beth Israel Deaconess Medical Center in Boston. Minocycline is ordinarily used to treat acne, but Trentham gave it to six patients with scleroderma and it effectively cured four of them (web). Scleroderma is a disease of excessive skin and internal organ fibrosis. The mechanism by which minocycline might alter this process is not known.
Minocycline has previously been reported to modestly improve joint symptoms in rheumatoid arthritis. Although this drug has a broad spectrum of antibiotic properties, the response of scleroderma patients does not necessarily implicate a microbial etiology to scleroderma. As with many other tetracycline derivatives, Minocycline has been documented to have many other pharmacologic properties, including inhibition of matrix metalloproteases. There have been a number of recent reports regarding the treatment of scleroderma with minocycline.
To investigate these reports, the Scleroderma Foundation contacted the researcher responsible for the study, as well as members of our Medical Advisory Board, and the American College of Rheumatology. The Scleroderma Foundation strongly supports research to find a cure. More than anyone else, the Foundation (patients, family members, treating physicians and researchers) knows the human cost of this disease. (web). Research is funded both privately and by the Federal government. Private Funding has currently raised over $2.
5 million raised and contributed to research each year. Although scleroderma affects a greater number of patients than MS, scleroderma research receives far less government support. In 1992, the National Institutes of Health provided $52. 7 million for MS research and only $3.
9 million for scleroderma research. Cystic Fibrosis, with only 25, 000 patients received $53. 1 million in that same year. (web). Through the years, many so-called "cures" for scleroderma have been put forward, but none have proven effective when given to a large number of patients in a scientific fashion. Scleroderma has many people thinking they have claimed a cure and it is really confusing to a lot of people.
Better therapies are being developed and a cure will eventually be found. Until then, we will use all our scientific resources to ensure that the information you receive is accurate, sound, reliable and true. To find a rheumatologist in your area, check the American College of Rheumatology membership directory. If you want more information on this or any other form of arthritis, contact the Arthritis Foundation at (800) 283-7800 or visit the Arthritis Foundation web site at web The Scleroderma Foundation can be reached at (800) 722-HOPE, or web.