Scleroderma

In a previous study. researchers sought to describe the medical history of a group of patients with Scleroderma Renal Crisis who had been treated with ACE inhibitors. The following is essentially a summary of that study, which was conducted by Virginia D. Steen, MD, and Thomas A. Medsger Jr., MD.

Scleroderma is a disease of unknown cause that produces fibrosis (hardening or scarring) of the connective tissue in the skin, blood vessels, and internal organs. These changes result in a tight, shiny appearance of the skin and potentially life-threatening abnormalities of the lungs, heart, digestive tract, and kidneys.

When Scleroderma involves the kidneys, it begins very suddenly with the onset of severely high blood pressure; this is called Scleroderma Renal Crisis. Without treatment, kidney failure requiring dialysis can develop and patients may die quickly. Dialysis involves connecting a person whose kidneys do not work to an artificial machine that filters waste products out of the blood.

The introduction more than 20 years ago of blood pressure drugs called angiotensin-converting enzyme (ACE) inhibitors has greatly improved the ability to treat Scleroderma Renal Crisis in the short term.

A group of researchers followed patients for up to 10 years after they had been treated with ACE inhibitors for Scleroderma Renal Crisis. Patients continued receiving ACE inhibitors after initial treatment. They also tried to identify factors that were related to better outcomes and determined patients' long-term outcomes.

Of the 145 patients studied with Scleroderma Renal Crisis, 89 had good long-term outcomes: 55 never required dialysis, and 34 (55% of those who initially required dialysis) had only temporary dialysis. Temporary dialysis lasted for an average of 8 months, and all patients were able to stop it by 18 months. Twenty-eight patients died within 6 months, and another 28 needed permanent dialysis.

This study included only patients treated at a single center, within a teaching hospital in Pittsburgh, Pennsylvania. Since patients treated with ACE inhibitors were not compared with others who did not receive this treatment, the study does not definitively prove that ACE inhibitors were the reason for the good outcomes. However, long-term outcomes were good in a substantial number of patients with Scleroderma Renal Crisis who were treated with ACE inhibitors.

For a link to Dr. Steen and Medsger's original and full article, do click here. For those of you who are logged in however, you can click on the link below to view the article directly.