Long-Term Outcomes of Scleroderma Renal Crisis

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The summary below is from the full report titled "Long-Term Outcomes of Scleroderma Renal Crisis." It is in the 17 October 2000 issue of Annals of Internal Medicine (volume 133, pages 600-603). The authors are VD Steen and TA Medsger Jr.

What is the problem and what is known about it so far?

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. There is little published information, however, on the long-term outcomes of patients with scleroderma renal crisis who were successfully treated with ACE inhibitors.

Why did the researchers do this particular study?

To describe the medical history of a group of patients with scleroderma renal crisis who had been treated with ACE inhibitors.

Who was studied?

145 patients treated at a scleroderma center in a teaching hospital in Pittsburgh, Pennsylvania.

How was the study done?

The 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. The researchers determined who died within 6 months of developing the condition, who required temporary dialysis, who required permanent dialysis, and who neither died within 6 months nor required dialysis. They also tried to identify factors that were related to better outcomes and determined patients' long-term outcomes.

What did the researchers find?

Of the 145 patients 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. Older men with heart disease were at greatest risk for permanent dialysis or death.

What were the limitations of the study?

This study included only patients treated at a single center. Since patients treated with ACE inhibitors were not compared with others who did not receive this treatment, the study does not definitely prove that ACE inhibitors were the reason for the good outcomes.

What are the implications of the study?

Long-term outcomes were good in a substantial number of patients with scleroderma renal crisis who were treated with ACE inhibitors.