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Related Experiment Videos

Scleroderma renal crisis: new insights and developments.

Elisa Y Rhew1, Walter G Barr

  • 1Department of Rheumatology, Feinberg School of Medicine, Northwestern University, 300 East Superior Street, Tarry 3-713, Chicago, IL 60611, USA. w-barr@northwestern.edu

Current Rheumatology Reports
|March 16, 2004
PubMed
Summary

Scleroderma renal crisis (SRC), a complication of systemic sclerosis (SSc), has improved outcomes with ACE inhibitors but remains serious. New therapies are needed to address ongoing mortality and morbidity from SRC.

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Area of Science:

  • Nephrology
  • Rheumatology
  • Internal Medicine

Background:

  • Scleroderma renal crisis (SRC) is a severe complication of systemic sclerosis (SSc).
  • Historically, SRC had a uniformly fatal outcome.
  • Significant improvements in outcomes have been observed with angiotensin-converting enzyme (ACE) inhibitors.

Purpose of the Study:

  • To review the epidemiology, pathogenesis, risk factors, clinical features, and treatment of SRC.
  • To highlight recent insights and developments in SRC management.
  • To emphasize the need for novel therapeutic strategies.

Main Methods:

  • Literature review of epidemiology, pathogenesis, risk factors, clinical features, and treatment of SRC.
  • Analysis of outcomes with current therapies, including ACE inhibitors.

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  • Discussion of pathophysiologic mechanisms and potential new treatments.

    • Main Results:

    • ACE inhibitors have significantly improved SRC outcomes.
    • Despite treatment, 39-50% of SSc patients with SRC still experience poor outcomes, including death or permanent dialysis.
    • Current treatments are not sufficient to prevent all severe morbidity and mortality.

    Conclusions:

    • Early recognition and ACE inhibitor treatment are crucial for managing SRC.
    • Novel therapies targeting underlying pathophysiologic mechanisms are essential for improving SRC outcomes.
    • Further research is needed to develop more effective treatments for SRC.