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

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 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

Access to Document

10.1007/s11926-015-0551-y

Cite this

@article{dc2d63baed6e4372b7c6836a7f4be55d,

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",

author = "Cybele Ghossein and John Varga and Fenves, {Andrew Z.}",

year = "2016",

month = jan,

day = "1",

doi = "10.1007/s11926-015-0551-y",

language = "English (US)",

volume = "18",

pages = "1--6",

journal = "Current Rheumatology Reports",

issn = "1523-3774",

publisher = "Current Science, Inc.",

number = "1",

}

TY - JOUR

T1 - Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis

AU - Ghossein, Cybele

AU - Varga, John

AU - Fenves, Andrew Z.

PY - 2016/1/1

Y1 - 2016/1/1

N2 - 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.

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.

KW - Angiotensin-converting enzyme inhibitor (ACEI)

KW - Diffuse cutaneous disease (dcSSc)

KW - Immune dysregulation

KW - Malignant hypertension

KW - Renal biopsy

KW - Scleroderma renal crisis (SRC)

KW - Systemic sclerosis

KW - Thrombotic microangiopathy

UR - http://www.scopus.com/inward/record.url?scp=84952013335&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84952013335&partnerID=8YFLogxK

U2 - 10.1007/s11926-015-0551-y

DO - 10.1007/s11926-015-0551-y

M3 - Review article

C2 - 26711696

AN - SCOPUS:84952013335

VL - 18

SP - 1

EP - 6

JO - Current Rheumatology Reports

JF - Current Rheumatology Reports

SN - 1523-3774

IS - 1

M1 - 5

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this