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Scleroderma renal crisis in progressive systemic sclerosis: a case report.

A Furukawa1, K Hashine, T Miyamoto

  • 1Department of Urology, Takamatsu Red Cross Hospital.

Hinyokika Kiyo. Acta Urologica Japonica
|December 1, 1990
PubMed
Summary

Systemic sclerosis can lead to scleroderma renal crisis, causing severe kidney dysfunction and hypertension unresponsive to treatment. This case highlights the critical, often fatal, progression of this rare complication.

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Area of Science:

  • Nephrology
  • Rheumatology
  • Internal Medicine

Background:

  • Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease characterized by fibrosis and vascular abnormalities.
  • Scleroderma renal crisis (SRC) is a serious complication of PSS, typically presenting with severe hypertension and acute kidney injury.
  • Early diagnosis and management are crucial for improving outcomes in SRC.

Observation:

  • A female patient with PSS developed SRC 10 years after disease onset.
  • The patient presented with progressive renal dysfunction, difficult-to-control hypertension, and massive gastrointestinal bleeding.
  • Standard antihypertensive treatments, including an angiotensin-converting enzyme inhibitor (enalapril), were ineffective.

Findings:

  • Intravenous nicardipine provided only a minimal effect on the patient's severe hypertension.

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  • Neither hemodialysis nor plasma exchange reversed the established renal crisis.
  • Kidney biopsy revealed thickened capillary walls and mild nephrosclerosis, indicative of vascular damage.

    • Implications:

    • This case underscores the aggressive and often fatal nature of SRC in advanced systemic sclerosis.
    • Refractory hypertension and renal failure in PSS patients necessitate prompt and aggressive management strategies.
    • Further research into novel therapeutic targets for SRC is warranted to improve patient survival and renal outcomes.