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Scleroderma Renal Crisis

MedGen UID:
853197
•Concept ID:
C1262147
•
Disease or Syndrome

Definition

The onset of acute renal failure, normally coupled with marked hypertension in a patient with scleroderma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVScleroderma Renal Crisis

Professional guidelines

PubMed

Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review.
Zhao M, Wu J, Wu H, Sawalha AH, Lu Q
Clin Rev Allergy Immunol 2022 Apr;62(2):273-291. Epub 2021 Jan 15 doi: 10.1007/s12016-020-08831-4. PMID: 33449302
Treatment Algorithms for Systemic Sclerosis According to Experts.
Fernández-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group
Arthritis Rheumatol 2018 Nov;70(11):1820-1828. Epub 2018 Sep 17 doi: 10.1002/art.40560. PMID: 29781586
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129

Recent clinical studies

Etiology

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.
Cole A, Ong VH, Denton CP
Clin Rev Allergy Immunol 2023 Jun;64(3):378-391. Epub 2022 Jun 1 doi: 10.1007/s12016-022-08945-x. PMID: 35648373Free PMC Article
Treatment Algorithms for Systemic Sclerosis According to Experts.
Fernández-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group
Arthritis Rheumatol 2018 Nov;70(11):1820-1828. Epub 2018 Sep 17 doi: 10.1002/art.40560. PMID: 29781586
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129
Scleroderma renal crisis.
Bose N, Chiesa-Vottero A, Chatterjee S
Semin Arthritis Rheum 2015 Jun;44(6):687-94. Epub 2014 Dec 11 doi: 10.1016/j.semarthrit.2014.12.001. PMID: 25613774
Scleroderma renal crisis.
Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L
J Rheumatol 2014 Jun;41(6):1040-8. Epub 2014 May 15 doi: 10.3899/jrheum.131210. PMID: 24833760

Diagnosis

Renal involvement in systemic sclerosis.
Scheen M, Dominati A, Olivier V, Nasr S, De Seigneux S, Mekinian A, Issa N, Haidar F
Autoimmun Rev 2023 Jun;22(6):103330. Epub 2023 Apr 7 doi: 10.1016/j.autrev.2023.103330. PMID: 37031831
Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.
Cole A, Ong VH, Denton CP
Clin Rev Allergy Immunol 2023 Jun;64(3):378-391. Epub 2022 Jun 1 doi: 10.1007/s12016-022-08945-x. PMID: 35648373Free PMC Article
Scleroderma renal crisis.
Hudson M, Ghossein C, Steen V
Presse Med 2021 Apr;50(1):104063. Epub 2021 Feb 3 doi: 10.1016/j.lpm.2021.104063. PMID: 33548376
Systemic sclerosis.
Denton CP, Khanna D
Lancet 2017 Oct 7;390(10103):1685-1699. Epub 2017 Apr 13 doi: 10.1016/S0140-6736(17)30933-9. PMID: 28413064
Scleroderma renal crisis.
Bose N, Chiesa-Vottero A, Chatterjee S
Semin Arthritis Rheum 2015 Jun;44(6):687-94. Epub 2014 Dec 11 doi: 10.1016/j.semarthrit.2014.12.001. PMID: 25613774

Therapy

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.
Cole A, Ong VH, Denton CP
Clin Rev Allergy Immunol 2023 Jun;64(3):378-391. Epub 2022 Jun 1 doi: 10.1007/s12016-022-08945-x. PMID: 35648373Free PMC Article
Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review.
Zhao M, Wu J, Wu H, Sawalha AH, Lu Q
Clin Rev Allergy Immunol 2022 Apr;62(2):273-291. Epub 2021 Jan 15 doi: 10.1007/s12016-020-08831-4. PMID: 33449302
Scleroderma renal crisis.
Hudson M, Ghossein C, Steen V
Presse Med 2021 Apr;50(1):104063. Epub 2021 Feb 3 doi: 10.1016/j.lpm.2021.104063. PMID: 33548376
Treatment Algorithms for Systemic Sclerosis According to Experts.
Fernández-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group
Arthritis Rheumatol 2018 Nov;70(11):1820-1828. Epub 2018 Sep 17 doi: 10.1002/art.40560. PMID: 29781586
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129

Prognosis

Management of scleroderma renal crisis.
Nagaraja V
Curr Opin Rheumatol 2019 May;31(3):223-230. doi: 10.1097/BOR.0000000000000604. PMID: 30870219
Therapy of scleroderma renal crisis: State of the art.
Zanatta E, Polito P, Favaro M, Larosa M, Marson P, Cozzi F, Doria A
Autoimmun Rev 2018 Sep;17(9):882-889. Epub 2018 Jul 10 doi: 10.1016/j.autrev.2018.03.012. PMID: 30005860
Scleroderma renal crisis and renal involvement in systemic sclerosis.
Woodworth TG, Suliman YA, Li W, Furst DE, Clements P
Nat Rev Nephrol 2016 Nov;12(11):678-691. Epub 2016 Sep 19 doi: 10.1038/nrneph.2016.124. PMID: 27641135
Scleroderma renal crisis.
Bose N, Chiesa-Vottero A, Chatterjee S
Semin Arthritis Rheum 2015 Jun;44(6):687-94. Epub 2014 Dec 11 doi: 10.1016/j.semarthrit.2014.12.001. PMID: 25613774
Scleroderma renal crisis.
Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L
J Rheumatol 2014 Jun;41(6):1040-8. Epub 2014 May 15 doi: 10.3899/jrheum.131210. PMID: 24833760

Clinical prediction guides

Renal involvement in systemic sclerosis.
Scheen M, Dominati A, Olivier V, Nasr S, De Seigneux S, Mekinian A, Issa N, Haidar F
Autoimmun Rev 2023 Jun;22(6):103330. Epub 2023 Apr 7 doi: 10.1016/j.autrev.2023.103330. PMID: 37031831
Systemic Sclerosis and Pulmonary Disease.
Ngo K
Adv Exp Med Biol 2021;1303:173-182. doi: 10.1007/978-3-030-63046-1_10. PMID: 33788193
Scleroderma renal crisis: observations from the South Australian Scleroderma Register.
Khadra Y, Walker JG, Hakendorf P, Proudman S, Rischmueller M, Hill CL, Whittle S, Pontifex E, Roberts-Thomson PJ
Intern Med J 2021 Feb;51(2):235-242. doi: 10.1111/imj.14772. PMID: 32043674
A New Paradigm for Renal Thrombotic Microangiopathy.
Gallan AJ, Chang A
Semin Diagn Pathol 2020 May;37(3):121-126. Epub 2020 Feb 6 doi: 10.1053/j.semdp.2020.01.002. PMID: 32085935
Treatment Algorithms for Systemic Sclerosis According to Experts.
Fernández-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group
Arthritis Rheumatol 2018 Nov;70(11):1820-1828. Epub 2018 Sep 17 doi: 10.1002/art.40560. PMID: 29781586

Recent systematic reviews

Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal.
Makris A, Panagiotopoulos A, Distler O, Sfikakis PP
J Rheumatol 2024 Nov 1;51(11):1060-1068. doi: 10.3899/jrheum.2023-1113. PMID: 38950948
Studying the Role of C5-Inhibition Therapy in Scleroderma Renal Crisis-Induced Thrombotic Microangiopathy - A Review of Literature.
Farrukh L, Steen VD, Shapiro L, Mehta S
Semin Arthritis Rheum 2023 Dec;63:152256. Epub 2023 Aug 22 doi: 10.1016/j.semarthrit.2023.152256. PMID: 37689027
Angiotensin-converting enzyme inhibitors prior to scleroderma renal crisis in systemic sclerosis: A systematic review and meta-analysis.
Xiong A, Cao Y, Xiang Q, Song Z, Zhang Y, Zhou S, Qiang Y, Chen H, Hu Z, Cui H, Luo J, Wang Y, Yang Y, Yang M, Shuai S
J Clin Pharm Ther 2022 Jun;47(6):722-731. Epub 2022 Mar 1 doi: 10.1111/jcpt.13621. PMID: 35233779
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129
Corticosteroids and the risk of scleroderma renal crisis: a systematic review.
Trang G, Steele R, Baron M, Hudson M
Rheumatol Int 2012 Mar;32(3):645-53. Epub 2010 Dec 4 doi: 10.1007/s00296-010-1697-6. PMID: 21132302

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