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 :

» What's Your Scleroderma Action Plan?

Living with any serious disease or condition like Scleroderma, is without a doubt, difficult. It is not hard to understand either, that we often can lose sight of what is important, including our way. We may also fall into to a false sense of...

» 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...

» Egr-1: A Target for Scleroderma Therapy

Two separate research groups funded by the (NIAMS) have discovered that the molecule EGR-1 (early growth response 1), which regulates gene expression, plays a central role in the development of fibrosis, a condition in which organ-supporting tissue...

» Research Highlights: Stem Cell Research

James White was in his 60's when he was diagnosed with , which progressed to. The traditional treatment for patients like White is standard , which brings a median survival time of about a year. But Duke oncologist David A. Rizzieri, MD, offered...

» Optical Coherence Tomography: The first Quantitative Imaging Biomarker for Scleroderma

Using the , manufactured in the UK by Michelson Diagnostics Ltd, scientists at the Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds have developed the first quantitative imaging biomarker for skin involvement in . Scleroderma...

» Vitamin D Deficiency May Hasten Autoimmune Lung Disease

Patients with autoimmune interstitial lung disease (ILD) — a frequently fatal chronic inflammatory autoimmune disease that causes scarring of the lungs — often have deficiencies, and researchers from the University of Cincinnati believe this...