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Rapidly progressive glomerulonephritis

MedGen UID:
113155
•Concept ID:
C0221239
•
Disease or Syndrome
Synonym: Glomerulonephritis, rapidly progressive
SNOMED CT: Rapidly progressive glomerulonephritis (236392004); RPGN - Rapidly progressive glomerulonephritis (236392004)
 
Monarch Initiative: MONDO:0017236

Definition

Inflammation of the glomeruli that is characterized by a rapid loss in renal function with glomerular crescent formation observed on biopsy; it is often seen in patients with concomitant autoimmune disease, like Goodpasture''s syndrome or systemic lupus erythematosus. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRapidly progressive glomerulonephritis
Follow this link to review classifications for Rapidly progressive glomerulonephritis in Orphanet.

Professional guidelines

PubMed

EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis.
Apaydin S
Transfus Apher Sci 2018 Feb;57(1):8-12. Epub 2018 Feb 20 doi: 10.1016/j.transci.2018.02.007. PMID: 29503131
Rapidly progressive crescentic glomerulonephritis: Early treatment is a must.
Moroni G, Ponticelli C
Autoimmun Rev 2014 Jul;13(7):723-9. Epub 2014 Mar 19 doi: 10.1016/j.autrev.2014.02.007. PMID: 24657897

Recent clinical studies

Etiology

IgA nephropathy.
Stamellou E, Seikrit C, Tang SCW, Boor P, Tesař V, Floege J, Barratt J, Kramann R
Nat Rev Dis Primers 2023 Nov 30;9(1):67. doi: 10.1038/s41572-023-00476-9. PMID: 38036542
Acute glomerulonephritis.
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
IgA Nephropathy: Core Curriculum 2021.
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Infection-Related Glomerulonephritis.
Hunt EAK, Somers MJG
Pediatr Clin North Am 2019 Feb;66(1):59-72. doi: 10.1016/j.pcl.2018.08.005. PMID: 30454751

Diagnosis

Anti-glomerular basement membrane vasculitis.
Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Acute Postinfectious Glomerulonephritis.
Duong MD, Reidy KJ
Pediatr Clin North Am 2022 Dec;69(6):1051-1078. Epub 2022 Oct 29 doi: 10.1016/j.pcl.2022.08.001. PMID: 36880922
Acute glomerulonephritis.
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
IgA Nephropathy: Core Curriculum 2021.
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Anti-Glomerular Basement Membrane Disease.
Gulati K, McAdoo SP
Rheum Dis Clin North Am 2018 Nov;44(4):651-673. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.011. PMID: 30274629

Therapy

EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Anti-glomerular basement membrane vasculitis.
Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Acute glomerulonephritis.
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
IgA Nephropathy: Core Curriculum 2021.
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311

Prognosis

IgA nephropathy.
Stamellou E, Seikrit C, Tang SCW, Boor P, Tesař V, Floege J, Barratt J, Kramann R
Nat Rev Dis Primers 2023 Nov 30;9(1):67. doi: 10.1038/s41572-023-00476-9. PMID: 38036542
IgA Nephropathy: Core Curriculum 2021.
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Post-infectious glomerulonephritis.
Balasubramanian R, Marks SD
Paediatr Int Child Health 2017 Nov;37(4):240-247. Epub 2017 Sep 11 doi: 10.1080/20469047.2017.1369642. PMID: 28891413
Histopathologic classification of ANCA-associated glomerulonephritis.
Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM
J Am Soc Nephrol 2010 Oct;21(10):1628-36. Epub 2010 Jul 8 doi: 10.1681/ASN.2010050477. PMID: 20616173

Clinical prediction guides

Predictors of rapidly progressive glomerulonephritis in acute poststreptococcal glomerulonephritis.
Karakaya D, Güngör T, Çakıcı EK, Yazılıtaş F, Çelikkaya E, Yücebaş SC, Bülbül M
Pediatr Nephrol 2023 Sep;38(9):3027-3033. Epub 2023 Mar 16 doi: 10.1007/s00467-023-05935-9. PMID: 36929388
Clinicohistological Profile and Outcomes of Rapidly Progressive Glomerulonephritis: A Prospective Study from North India.
Sharma D, Mehta S, Sandhu JS, Sohal PM, Makkar V
Saudi J Kidney Dis Transpl 2022 Jan-Feb;33(1):122-131. doi: 10.4103/1319-2442.367805. PMID: 36647986
Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge.
Ai S, Liu J, Ma G, Ye W, Hu R, Zhang S, Fan X, Liu B, Miao Q, Qin Y, Li X
Ann Med 2022 Dec;54(1):754-763. doi: 10.1080/07853890.2022.2046288. PMID: 35243934Free PMC Article
IgA Nephropathy: Core Curriculum 2021.
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Histopathologic classification of ANCA-associated glomerulonephritis.
Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM
J Am Soc Nephrol 2010 Oct;21(10):1628-36. Epub 2010 Jul 8 doi: 10.1681/ASN.2010050477. PMID: 20616173

Recent systematic reviews

EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Efficacy and Safety of Plasma Exchange as an Adjunctive Therapy for Rapidly Progressive IgA Nephropathy and Henoch-Schönlein Purpura Nephritis: A Systematic Review.
Nguyen B, Acharya C, Tangpanithandee S, Miao J, Krisanapan P, Thongprayoon C, Amir O, Mao MA, Cheungpasitporn W, Acharya PC
Int J Mol Sci 2023 Feb 16;24(4) doi: 10.3390/ijms24043977. PMID: 36835388Free PMC Article
The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis.
Walsh M, Collister D, Zeng L, Merkel PA, Pusey CD, Guyatt G, Au Peh C, Szpirt W, Ito-Hara T, Jayne DRW; Plasma exchange and glucocorticoid dosing for patients with ANCA-associated vasculitis BMJ Rapid Recommendations Group
BMJ 2022 Feb 25;376:e064604. doi: 10.1136/bmj-2021-064604. PMID: 35217545
Interventions for renal vasculitis in adults.
Walters G, Willis NS, Craig JC
Cochrane Database Syst Rev 2015 Sep 24;(9):CD003232. doi: 10.1002/14651858.CD003232.pub3. PMID: 26400765
Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis.
Walsh M, Catapano F, Szpirt W, Thorlund K, Bruchfeld A, Guillevin L, Haubitz M, Merkel PA, Peh CA, Pusey C, Jayne D
Am J Kidney Dis 2011 Apr;57(4):566-74. Epub 2010 Dec 30 doi: 10.1053/j.ajkd.2010.10.049. PMID: 21194817Free PMC Article

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