U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Renal vasculitis

MedGen UID:
508849
•Concept ID:
C0152002
•
Disease or Syndrome
SNOMED CT: Renal vasculitis (95578000)
 
HPO: HP:6000076

Definition

Vasculitis (inflammation of blood vessels) in the kidney. Renal vasculitis can be observed with numerous disorders and presents clinically as rapidly progressive glomerulonephritis (RPGN) which is characterized by acute kidney injury (AKI), haematuria and proteinuria. Histological examination of the kidney reveals severe inflammation in the form of crescent formation, glomerular necrosis and vasculitis of small and medium sized vessels within the kidney. [from HPO]

Professional guidelines

PubMed

An update on dıagnosıs and treatment of ANCA assocıated renal vasculıtıs.
Turkmen K, Ozer H, Tesar V
Int Urol Nephrol 2023 Nov;55(11):2817-2827. Epub 2023 Apr 3 doi: 10.1007/s11255-023-03565-6. PMID: 37010734
Utility of renal biopsy in the clinical management of renal disease.
Dhaun N, Bellamy CO, Cattran DC, Kluth DC
Kidney Int 2014 May;85(5):1039-48. Epub 2014 Jan 8 doi: 10.1038/ki.2013.512. PMID: 24402095
Current treatment strategies in ANCA-positive renal vasculitis-lessons from European randomized trials.
Tesar V, Ríhová Z, Jancová E, Rysavá R, Merta M; European randomized trials
Nephrol Dial Transplant 2003 Jul;18 Suppl 5:v2-4. doi: 10.1093/ndt/gfg1032. PMID: 12817056

Recent clinical studies

Etiology

Efficacy and safety of avacopan in patients with ANCA-associated vasculitis receiving rituximab in a randomised trial.
Geetha D, Dua A, Yue H, Springer J, Salvarani C, Jayne D, Merkel P; ADVOCATE Study Group
Ann Rheum Dis 2024 Jan 11;83(2):223-232. doi: 10.1136/ard-2023-224816. PMID: 37979959Free PMC Article
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial.
Jones RB, Furuta S, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh M, Westman K, Jayne DR; European Vasculitis Society (EUVAS)
Ann Rheum Dis 2015 Jun;74(6):1178-82. Epub 2015 Mar 4 doi: 10.1136/annrheumdis-2014-206404. PMID: 25739829
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh D, Walsh M, Westman K, Jayne DR; European Vasculitis Study Group
N Engl J Med 2010 Jul 15;363(3):211-20. doi: 10.1056/NEJMoa0909169. PMID: 20647198
Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.
Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage CO, Sinico RA, Stegeman CA, Westman KW, van der Woude FJ, de Lind van Wijngaarden RA, Pusey CD; European Vasculitis Study Group
J Am Soc Nephrol 2007 Jul;18(7):2180-8. Epub 2007 Jun 20 doi: 10.1681/ASN.2007010090. PMID: 17582159

Diagnosis

Efficacy and safety of avacopan in patients with ANCA-associated vasculitis receiving rituximab in a randomised trial.
Geetha D, Dua A, Yue H, Springer J, Salvarani C, Jayne D, Merkel P; ADVOCATE Study Group
Ann Rheum Dis 2024 Jan 11;83(2):223-232. doi: 10.1136/ard-2023-224816. PMID: 37979959Free PMC Article
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial.
Jones RB, Furuta S, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh M, Westman K, Jayne DR; European Vasculitis Society (EUVAS)
Ann Rheum Dis 2015 Jun;74(6):1178-82. Epub 2015 Mar 4 doi: 10.1136/annrheumdis-2014-206404. PMID: 25739829
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh D, Walsh M, Westman K, Jayne DR; European Vasculitis Study Group
N Engl J Med 2010 Jul 15;363(3):211-20. doi: 10.1056/NEJMoa0909169. PMID: 20647198
Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.
Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage CO, Sinico RA, Stegeman CA, Westman KW, van der Woude FJ, de Lind van Wijngaarden RA, Pusey CD; European Vasculitis Study Group
J Am Soc Nephrol 2007 Jul;18(7):2180-8. Epub 2007 Jun 20 doi: 10.1681/ASN.2007010090. PMID: 17582159

Therapy

Efficacy and safety of avacopan in patients with ANCA-associated vasculitis receiving rituximab in a randomised trial.
Geetha D, Dua A, Yue H, Springer J, Salvarani C, Jayne D, Merkel P; ADVOCATE Study Group
Ann Rheum Dis 2024 Jan 11;83(2):223-232. doi: 10.1136/ard-2023-224816. PMID: 37979959Free PMC Article
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial.
Jones RB, Furuta S, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh M, Westman K, Jayne DR; European Vasculitis Society (EUVAS)
Ann Rheum Dis 2015 Jun;74(6):1178-82. Epub 2015 Mar 4 doi: 10.1136/annrheumdis-2014-206404. PMID: 25739829
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh D, Walsh M, Westman K, Jayne DR; European Vasculitis Study Group
N Engl J Med 2010 Jul 15;363(3):211-20. doi: 10.1056/NEJMoa0909169. PMID: 20647198
Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.
Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage CO, Sinico RA, Stegeman CA, Westman KW, van der Woude FJ, de Lind van Wijngaarden RA, Pusey CD; European Vasculitis Study Group
J Am Soc Nephrol 2007 Jul;18(7):2180-8. Epub 2007 Jun 20 doi: 10.1681/ASN.2007010090. PMID: 17582159

Prognosis

NETosis: an emerging therapeutic target in renal diseases.
Juha M, Molnár A, Jakus Z, Ledó N
Front Immunol 2023;14:1253667. Epub 2023 Sep 8 doi: 10.3389/fimmu.2023.1253667. PMID: 37744367Free PMC Article
Vasculitis and Kidney Disease.
Grewal MK, Adams MD, Valentini RP
Pediatr Clin North Am 2022 Dec;69(6):1199-1217. Epub 2022 Oct 29 doi: 10.1016/j.pcl.2022.07.009. PMID: 36880930
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, Savage CO, Segelmark M, Tesar V, van Paassen P, Walsh D, Walsh M, Westman K, Jayne DR; European Vasculitis Study Group
N Engl J Med 2010 Jul 15;363(3):211-20. doi: 10.1056/NEJMoa0909169. PMID: 20647198
Renal vasculitis.
D'Amico G, Sinico RA, Ferrario F
Nephrol Dial Transplant 1996;11 Suppl 9:69-74. doi: 10.1093/ndt/11.supp9.69. PMID: 9050038

Clinical prediction guides

Evaluating renal outcome of ANCA-associated renal vasculitis: comparative study of two histopathological scoring systems.
An XN, Wei ZN, Yao XY, Xu J, Qian WT, Pan XX, Shen PY, Shi H, Zhang W, Chen XN, Chen N, Chen YX
Clin Exp Rheumatol 2021 Mar-Apr;39 Suppl 129(2):39-45. Epub 2020 Dec 9 doi: 10.55563/clinexprheumatol/24ep0c. PMID: 33337988
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Vasculitis update: pathogenesis and biomarkers.
Brogan P, Eleftheriou D
Pediatr Nephrol 2018 Feb;33(2):187-198. Epub 2017 Aug 7 doi: 10.1007/s00467-017-3597-4. PMID: 28785984Free PMC Article
ANCA-associated vasculitis with renal involvement.
Binda V, Moroni G, Messa P
J Nephrol 2018 Apr;31(2):197-208. Epub 2017 May 30 doi: 10.1007/s40620-017-0412-z. PMID: 28560688
Repeat protocol renal biopsy in ANCA-associated renal vasculitis.
Hruskova Z, Honsova E, Berden AE, Rychlik I, Lanska V, Zabka J, Bajema IM, Tesar V
Nephrol Dial Transplant 2014 Sep;29(9):1728-32. Epub 2014 Feb 26 doi: 10.1093/ndt/gfu042. PMID: 24578468

Recent systematic reviews

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.
Corthier A, Jachiet M, Bertin D, Servais A, Barbet C, Bigot A, Doutre MS, Bessis D, Bouffandeau A, Moranne O, Jarrot PA, Bardin N, Terrier B, Burtey S, Puéchal X, Daniel L, Jourde-Chiche N
BMC Nephrol 2022 Feb 16;23(1):67. doi: 10.1186/s12882-022-02689-8. PMID: 35172758Free PMC Article
Role of therapeutic plasmapheresis in ANCA-associated vasculitis.
Walters G
Pediatr Nephrol 2016 Feb;31(2):217-25. Epub 2015 May 19 doi: 10.1007/s00467-014-3038-6. PMID: 25986911
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
Interventions for renal vasculitis in adults. A systematic review.
Walters GD, Willis NS, Craig JC
BMC Nephrol 2010 Jun 24;11:12. doi: 10.1186/1471-2369-11-12. PMID: 20573267Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...