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Scleroderma renal crisis.

Nilanjana Bose1, Andres Chiesa-Vottero1, Soumya Chatterjee1

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Summary
This summary is machine-generated.

Scleroderma renal crisis (SRC) is a severe complication of scleroderma. Prompt diagnosis and ACE inhibitor treatment have significantly improved survival rates for SRC patients.

Keywords:
Angiotensin-converting enzyme inhibitorSclerodermaScleroderma renal crisisSystemic sclerosis

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Area of Science:

  • Nephrology
  • Rheumatology
  • Internal Medicine

Background:

  • Scleroderma renal crisis (SRC) is a critical complication of scleroderma (systemic sclerosis).
  • SRC affects approximately 10% of scleroderma patients, presenting with malignant hypertension and renal failure.
  • Normotensive SRC occurs in about 10% of cases.

Purpose of the Study:

  • To comprehensively review the pathophysiology, risk factors, clinical features, diagnosis, treatment, prevention, and outcomes of SRC.
  • To highlight the impact of modern therapies on SRC prognosis.

Main Methods:

  • Literature search of PubMed using keywords: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, prognosis.
  • Systematic review and assessment of risk factors, treatment options, prognostic factors, and survival data.

Main Results:

  • SRC pathogenesis involves renal arteriole injury, leading to hypertension and renal failure.
  • Risk factors include rapid skin thickening, specific medications, anemia, thrombocytopenia, cardiac issues, and anti-RNA polymerase III antibodies.
  • Angiotensin-converting enzyme (ACE) inhibitors have reduced SRC mortality from 76% to under 10% since the 1970s.

Conclusions:

  • SRC remains a significant cause of morbidity and mortality in scleroderma.
  • Prognosis has dramatically improved with ACE inhibitors, emphasizing the need for prompt diagnosis and treatment.
  • Renal transplantation offers improved survival, though SRC recurrence is possible.