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 in | Modern rheumatology Vol. 33; no. 1; pp. 12 - 20 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
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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. |
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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 |
Author_xml | – sequence: 1 givenname: Chingching orcidid: 0000-0002-1964-4389 surname: Foocharoen fullname: Foocharoen, Chingching organization: Department of Medicine, Division of Rheumatology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand – sequence: 2 givenname: Pantipa surname: Tonsawan fullname: Tonsawan, Pantipa organization: Department of Medicine, Division of Nephrology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand – sequence: 3 givenname: Patnarin surname: Pongkulkiat fullname: Pongkulkiat, Patnarin organization: Department of Medicine, Division of Rheumatology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand – sequence: 4 givenname: Sirirat surname: Anutrakulchai fullname: Anutrakulchai, Sirirat organization: Department of Medicine, Division of Nephrology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand – sequence: 5 givenname: Ajanee surname: Mahakkanukrauh fullname: Mahakkanukrauh, Ajanee organization: Department of Medicine, Division of Rheumatology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand – sequence: 6 givenname: Siraphop surname: Suwannaroj fullname: Suwannaroj, Siraphop organization: Department of Medicine, Division of Rheumatology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/35349704$$D View this record in MEDLINE/PubMed |
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CitedBy_id | crossref_primary_10_1016_j_semarthrit_2023_152256 crossref_primary_10_2169_internalmedicine_2970_23 crossref_primary_10_1007_s11560_023_00690_2 |
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Keywords | thrombotic microangiopathy vasculopathy renal crisis Systemic sclerosis scleroderma |
Language | English |
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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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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 |
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