• Curr Rheumatol Rep · Jan 2016

    Review

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

    • Cybele Ghossein, John Varga, and Andrew Z Fenves.
    • Department of Medicine, Division of Nephrology, Northwestern University, Feinberg School of Medicine, 710 North Fairbanks Court-Suite 4500, Chicago, IL, 60611, USA. c-ghossein@northwestern.edu.
    • Curr Rheumatol Rep. 2016 Jan 1; 18 (1): 5.

    AbstractScleroderma 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.

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