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

Cybele Ghossein; John Varga; Andrew Z. Fenves

doi:10.1007/s11926-015-0551-y

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 journal › Review article › peer-review

10 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 language	English (US)
Article number	5
Pages (from-to)	1-6
Number of pages	6
Journal	Current rheumatology reports
Volume	18
Issue number	1
DOIs	https://doi.org/10.1007/s11926-015-0551-y
State	Published - 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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title = "Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis",

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.",

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

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AB - 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.

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KW - Thrombotic microangiopathy

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