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 :

» Analytical Chemistry Used In Systemic Sclerosis Study

Scientists have used methods to identify new target autoantigens in patients with systemic sclerosis (SSc).The study, published by , stated that antinuclear antibodies (ANA) can normally be found in 90 per cent of SSc patients with the use of...

» What Is Scleroderma?

DefinitionDerived from the Greek words “sklerosis,” meaning hardness, and “derma,” meaning skin, Scleroderma literally means hard skin. Although it is often referred to as if it were a single disease, Scleroderma is really a symptom of a...

» 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...

» Role of ACE Inhibitors in Preventing Scleroderma Renal Crisis Remains Unclear

Scleroderma patients on angiotensin-converting enzyme inhibitors at the onset of Scleroderma renal crisis do not appear to have worse outcomes than do those not taking the antihypertensive agents prior to the acute renal function deterioration,...

» Scientists and Clinicians Address Diagnosis and Treatment for Autoimmune and Autoinflammatory Diseases

Autoimmune and autoinflammatory diseases are a major health issue. The National Institutes of Health reports that up to 23.5 million Americans are afflicted. The American Autoimmune Related Diseases Association reports the number is closer to 50...

» 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...