Management review of scleroderma renal crisis: An update with practical pointers

Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called angiotensin-converting enzyme inhibitor...

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Published inModern rheumatology Vol. 33; no. 1; pp. 12 - 20
Main Authors Foocharoen, Chingching, Tonsawan, Pantipa, Pongkulkiat, Patnarin, Anutrakulchai, Sirirat, Mahakkanukrauh, Ajanee, Suwannaroj, Siraphop
Format Journal Article
LanguageEnglish
Published England 03.01.2023
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Abstract Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called angiotensin-converting enzyme inhibitor is the treatment of choice. The efficacy of various other vasodilators (i.e. endothelin-1 receptor antagonist) and complement cascade blocker for SRC have been investigated; however, no randomized control trial has been conducted. A new approach has been proposed for the management of SRC, categorized by specific clinical features of narrowly defined SRC and systemic sclerosis-thrombotic microangiopathy. SRC prophylaxis using angiotensin-converting enzyme inhibitor might be harmful, leading to a poor renal outcome, so the pathogenesis of SRC needs to be clarified in order to identify other possible preventions or therapies.
AbstractList ABSTRACT Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called angiotensin-converting enzyme inhibitor is the treatment of choice. The efficacy of various other vasodilators (i.e. endothelin-1 receptor antagonist) and complement cascade blocker for SRC have been investigated; however, no randomized control trial has been conducted. A new approach has been proposed for the management of SRC, categorized by specific clinical features of narrowly defined SRC and systemic sclerosis-thrombotic microangiopathy. SRC prophylaxis using angiotensin-converting enzyme inhibitor might be harmful, leading to a poor renal outcome, so the pathogenesis of SRC needs to be clarified in order to identify other possible preventions or therapies.
Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called angiotensin-converting enzyme inhibitor is the treatment of choice. The efficacy of various other vasodilators (i.e. endothelin-1 receptor antagonist) and complement cascade blocker for SRC have been investigated; however, no randomized control trial has been conducted. A new approach has been proposed for the management of SRC, categorized by specific clinical features of narrowly defined SRC and systemic sclerosis-thrombotic microangiopathy. SRC prophylaxis using angiotensin-converting enzyme inhibitor might be harmful, leading to a poor renal outcome, so the pathogenesis of SRC needs to be clarified in order to identify other possible preventions or therapies.
Author Mahakkanukrauh, Ajanee
Pongkulkiat, Patnarin
Foocharoen, Chingching
Anutrakulchai, Sirirat
Tonsawan, Pantipa
Suwannaroj, Siraphop
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crossref_primary_10_2169_internalmedicine_2970_23
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Keywords thrombotic microangiopathy
vasculopathy
renal crisis
Systemic sclerosis
scleroderma
Language English
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SSID ssj0035989
Score 2.3736162
Snippet Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy...
ABSTRACT Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement...
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StartPage 12
SubjectTerms Acute Kidney Injury - etiology
Acute Kidney Injury - pathology
Acute Kidney Injury - therapy
Angiotensin-Converting Enzyme Inhibitors - therapeutic use
Humans
Kidney - pathology
Scleroderma, Localized
Scleroderma, Systemic - complications
Scleroderma, Systemic - drug therapy
Scleroderma, Systemic - pathology
Title Management review of scleroderma renal crisis: An update with practical pointers
URI https://www.ncbi.nlm.nih.gov/pubmed/35349704
https://search.proquest.com/docview/2645470566
Volume 33
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