Long-Term Outcomes of Scleroderma Renal Crisis PDF Print E-mail
Wednesday, 17 March 2010 13:59
Virginia D. Steen, MD, and Thomas A. Medsger Jr., MD
Published: October 17th, 2000

Background: Although scleroderma renal crisis, a complication of systemic sclerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitors, its long-term outcomes are not known.

Objective: To determine outcomes, natural history, and risk factors in patients with systemic sclerosis and scleroderma renal crisis.

Design: Prospective observational cohort study.

Setting: University program specializing in scleroderma.

Patients: 145 patients with scleroderma renal crisis who received ACE inhibitors and 662 patients with scleroderma who did not have renal crisis.

Measurements: Among patients with renal crisis, the four outcomes studied were no dialysis, temporary dialysis, permanent dialysis, and early death. Demographic, clinical, and laboratory data were compared to identify risk factors for specific outcomes. Follow-up was 5 to 10 years.

Results: 61% of patients with renal crisis had good outcomes (55 received no dialysis, and 34 received temporary dialysis); only 4 of these (4%) progressed to chronic renal failure and permanent dialysis. More than half of the patients who initially required dialysis could discontinue it 3 to 18 months later. Survival of patients in the good outcome group was similar to that of patients with diffuse scleroderma who did not have renal crisis. Some patients (39%) had bad outcomes (permanent dialysis or early death). Conclusions: Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis.

Scleroderma renal crisis (1) is defined as the new onset of severe hypertension associated with a rapid increase in serum creatinine concentration, microangiopathic hemolytic anemia, or both. Outcomes of this disorder, a once-fatal complication of scleroderma, have dramatically improved with the use of angiotensin-converting enzyme (ACE) inhibitors (1). We examined short- and long-term outcomes
of renal crisis in patients taking these medications...

To continue reading the full article, please login in and download from the link provided below.

 
More articles :

» Endothelin Antagonists Present Great Promise

One of the most intriguing developments in recent medical science is the discovery of the human chemical endothelin (ET). Since its detection in 1988, over 22,000 scholarly articles (about 3 per day) have been published on the subject, a new class...

» Biomarkers Predict Lung Decline in Systemic Sclerosis

Predicting risk of pulmonary fibrosis and pulmonary hypertension is an urgent priority in systemic sclerosis. Lung function often declines rapidly in the first years after diagnosis, and interstitial lung disease (ILD), although highly variable in...

» Liver Autoantibodies in Patients with Scleroderma

Thelma L. Skare & Renato M. Nisihara & Osvaldo Haider & Pedro M. Azevedo & Shirley R. R. UtiyamaClin RheumatolDOI 10.1007/s10067-010-1586-0Association between autoimmune liver diseases and scleroderma has been described. The purpose...

» What is Chelation Therapy?

Chelation (pronounced key-LAY-shun) therapy is a chemical process in which a synthetic solution-EDTA (ethylenediaminetetraacetic acid) is injected into the bloodstream to remove heavy metals and/or minerals from the body. Chelation means "to grab"...

» Cancer Drug May Also Work for Scleroderma

A drug used to treat cancer may also be effective in diseases that cause scarring of the internal organs or skin, such as pulmonary fibrosis or Scleroderma.The drug, with the generic name bortezomib, stopped the production of fibrotic proteins in...

» Survival In Pulmonary Hypertension

Stephen C. Mathai, Laura K. Hummers, Hunter C. Champion, Fredrick M. Wigley, Ari Zaiman, Paul M. Hassoun, and Reda E. GirgisARTHRITIS & RHEUMATISMVol. 60, No. 2, February 2009, pp 569–577 DOI 10.1002/art.24267 © 2009, American College of...