Scleroderma

  • Medical Author:
    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.

  • Medical Editor: Charles Patrick Davis, MD, PhD
    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.

View the Rheumatoid Arthritis Slideshow

What is the prognosis (outlook) for patients with scleroderma?

A patient's prognosis is optimized with close monitoring of overall health status and treatment of complications, especially elevated blood pressure. This can involve medical subspecialists, including rheumatologists, pulmonologists, gastroenterologists, nephrologists, and cardiologists. Research indicates that the critical period of organ risk is generally within the first three years of skin involvement. This means that patients can be reassured that their risk of organ-threatening complications is significantly less after three years of having skin symptoms and not having 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.

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. Ongoing research into new medications for pulmonary hypertension are in clinical trials. Researchers are also evaluating the possible role of stem-cell transplantation for certain serious forms of 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. Many clinical research trials are under way to evaluate 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.

Medically Reviewed by a Doctor on 3/18/2016

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