Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis

Cybele Ghossein*, John Varga, Andrew Z. Fenves

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

15 Scopus citations

Abstract

Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.

Original languageEnglish (US)
Article number5
Pages (from-to)1-6
Number of pages6
JournalCurrent rheumatology reports
Volume18
Issue number1
DOIs
StatePublished - Jan 1 2016

Keywords

  • Angiotensin-converting enzyme inhibitor (ACEI)
  • Diffuse cutaneous disease (dcSSc)
  • Immune dysregulation
  • Malignant hypertension
  • Renal biopsy
  • Scleroderma renal crisis (SRC)
  • Systemic sclerosis
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Rheumatology

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