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Scleroderma

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.

Alternative Names

Systemic sclerosis

Highlights

Overview:

The name scleroderma comes from the Greek words skleros , which means hard, and derma , meaning skin. The disease is categorized as a rheumatologic disorder because it affects the connective tissues in the body.

Treatment News:

• Results from the Scleroderma Lung Study show that cyclophosphamide, a drug used to suppress the immune system, improves the lung condition of scleroderma patients with lung complications. The improvements lasted for 2 years (the duration of the study).
• A Phase I trial of reduced-intensity stem cell transplant showed the procedure is safe and effective. Patients' skin improved significantly, and their heart, lung, and kidney functions remained stable during the 12-month study.

Current Research:

• Scientists found that antibodies often present in patients with scleroderma and systemic lupus erythematosus (SLE) bind to different parts of a single protein. Scientists hope this finding will one day lead to a specific diagnostic test for scleroderma.
• Researchers now know that cells in the blood vessels and skin of scleroderma patients make too much of certain chemicals, and not enough of others. Studies revealed that the cause is an alteration in the DNA, the hereditary material. These changes "turn off" some genes and "turn up" others. It is hoped that certain drugs, some already used in cancer treatments, can some day be used to stop these DNA changes.
• In mice, scientists found that stopping a chemical messenger called TGF-beta halted a scleroderma-like condition in the animals.

• Review Date: 12/14/2006
• Reviewed By: Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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