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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
Is there today a place for corticosteroids in the treatment of scleroderma?
Blagojevic J, Legendre P, Matucci-Cerinic M, Mouthon L
Autoimmun Rev 2019 Dec;18(12):102403. Epub 2019 Oct 19 doi: 10.1016/j.autrev.2019.102403. PMID: 31639515
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
Is there today a place for corticosteroids in the treatment of scleroderma?
Blagojevic J, Legendre P, Matucci-Cerinic M, Mouthon L
Autoimmun Rev 2019 Dec;18(12):102403. Epub 2019 Oct 19 doi: 10.1016/j.autrev.2019.102403. PMID: 31639515
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 in Myositis and Scleroderma.
Postolova A, Chen JK, Chung L
Rheum Dis Clin North Am 2016 Feb;42(1):103-18, ix. Epub 2015 Oct 26 doi: 10.1016/j.rdc.2015.08.011. PMID: 26611554Free PMC Article
The scleroderma kidney: progress in risk factors, therapy, and prevention.
Bussone G, Bérezné A, Pestre V, Guillevin L, Mouthon L
Curr Rheumatol Rep 2011 Feb;13(1):37-43. doi: 10.1007/s11926-010-0145-7. PMID: 21061100

Diagnosis

Respiratory failure in systemic sclerosis.
Landim JIVD, Franco AS, Sampaio-Barros PD, Miossi R, Medeiros-Ribeiro AC, Pereira RMR, Assad APL
Rheumatol Int 2024 Nov;44(11):2653-2658. Epub 2023 Oct 14 doi: 10.1007/s00296-023-05482-4. PMID: 37837449
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
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
Corticosteroids in Myositis and Scleroderma.
Postolova A, Chen JK, Chung L
Rheum Dis Clin North Am 2016 Feb;42(1):103-18, ix. Epub 2015 Oct 26 doi: 10.1016/j.rdc.2015.08.011. PMID: 26611554Free PMC Article
Assessment of kidney involvement.
Steen VD, Mayes MD, Merkel PA
Clin Exp Rheumatol 2003;21(3 Suppl 29):S29-31. PMID: 12889219

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
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 in Myositis and Scleroderma.
Postolova A, Chen JK, Chung L
Rheum Dis Clin North Am 2016 Feb;42(1):103-18, ix. Epub 2015 Oct 26 doi: 10.1016/j.rdc.2015.08.011. PMID: 26611554Free PMC Article
Treatment of systemic sclerosis.
Pope JE
Rheum Dis Clin North Am 1996 Nov;22(4):893-907. doi: 10.1016/s0889-857x(05)70307-0. PMID: 8923602

Prognosis

Autoantibodies Targeting G-Protein-Coupled Receptors: Pathogenetic, Clinical and Therapeutic Implications in Systemic Sclerosis.
Binda M, Moccaldi B, Civieri G, Cuberli A, Doria A, Tona F, Zanatta E
Int J Mol Sci 2024 Feb 15;25(4) doi: 10.3390/ijms25042299. PMID: 38396976Free PMC Article
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.
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
The scleroderma kidney: progress in risk factors, therapy, and prevention.
Bussone G, Bérezné A, Pestre V, Guillevin L, Mouthon L
Curr Rheumatol Rep 2011 Feb;13(1):37-43. doi: 10.1007/s11926-010-0145-7. PMID: 21061100
Scleroderma and related conditions.
Mitchell H, Bolster MB, LeRoy EC
Med Clin North Am 1997 Jan;81(1):129-49. doi: 10.1016/s0025-7125(05)70508-3. PMID: 9012758

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
Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement.
Shimizu T, Saito C, Watanabe M, Ishii R, Kawamura T, Nagai K, Fujita A, Kaneko S, Kai H, Morito N, Usui J, Yokosawa M, Kondo Y, Inoue S, Okiyama N, Yamagata K
Intern Med 2021 Apr 1;60(7):1101-1107. Epub 2020 Nov 2 doi: 10.2169/internalmedicine.5665-20. PMID: 33132335Free PMC Article
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
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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