Long-Term Outcomes of Scleroderma Renal Crisis PDF Print E-mail
Monday, 14 December 2009 20:34
taken from http://www.flickr.com/photos/duncan/104321008/ via creative commonsIn 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.
 
More articles :

» A Brief History of Scleroderma

While scleroderma may not be very well known to the average person, investigation reveals it has been around for a long time.Classical ReferencesCases of skin disease similar to scleroderma may be found in the writings of Hippocrates as far back as...

» Scleroderma Renal Crisis: A Pathology Perspective

Ibrahim Batal, Robyn T. Domsic, Thomas A.Medsger Jr., and Sheldon BastackyInternational Journal of RheumatologyVolume 2010, Article ID 543704Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is...

» Silicone and Scleroderma Revisited

Silicone, a synthetic polymer considered to be a biologically inert substance, is used in a multitude of medical products, the most publicly recognized of which are breast implants. Silicone breast implants have been in use since the early 1960s for...

» Teen To Graduate, Despite Fatal Disease

A rare and life threatening medical disorder has kept a Portsmouth teen out of school and unable to walk for months, but Wednesday night he will walk the stage at his graduation. Wednesday night, families will be filing into the N-Telos pavilion in...

» Predictors Of Interstitial Lung Disease In Early Systemic Sclerosis

Introduction: The objective of the present study was to examine the association of baseline demographic andclinical characteristics with sequentially obtained measurements of forced vital capacity (FVC), expressed as apercentage of the predicted...

» New Study Will Explore Impact of Exercise on Pulmonary Hypertension

For sufferers of Pulmonary Arterial Hypertension (PAH), maintaining healthy heart function isn't as simple as going for a jog every morning. Patients need to do all they can to slow damage to their heart, and exercise can improve potentially improve...