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Medical Author:

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.

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Medical Editor:

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.

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In this Article

• Scleroderma facts
• What is scleroderma?
• What causes scleroderma?
• What are risk factors for developing scleroderma?
• How is scleroderma classified?
• What are scleroderma symptoms and signs?
• How is scleroderma diagnosed?
• What is the treatment for scleroderma?
• What is the prognosis (outlook) for patients with scleroderma?
• Can scleroderma be prevented?
• Patient Comments: Scleroderma - Symptoms and Signs
• Patient Comments: Scleroderma - Describe Your Experience
• Patient Comments: Scleroderma - Treatment
• Find a local Rheumatologist in your town
• Scleroderma Index

A patient's prognosis is optimized with close monitoring of overall health status and treatment of complications, especially elevated blood pressure. Research indicates that the critical period of organ risk is generally within the first 3 years of skin involvement. This means that patients can be reassured that their risk of organ-threatening complications is significantly less after 3 years of having skin symptoms and no internal organ problems.

Patients with diffuse skin involvement tend to do worse than those with limited skin involvement. Those with limited disease of the skin can develop serious lung scarring and pulmonary artery hypertension even after a decade of disease. Lung disease is the leading cause of death. Those with poor pulmonary function tests can have worse outcomes.

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 under way that are evaluating potential treatments for various aspects of the disease to improve the prognosis for patients with scleroderma. Further research supported by the Scleroderma Foundation, Arthritis Foundation, and the National Institutes of Health will lead to better understanding the illness and more optimal care for patients with scleroderma.