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Anti-neutrophil cytoplasmic antibody-associated vasculitis

MedGen UID:
403453
Concept ID:
C2717865
Disease or Syndrome
Synonyms: ANCA Associated Vasculitides; ANCA Associated Vasculitis; ANCA-Associated Vasculitide; ANCA-Associated Vasculitides; ANCA-Associated Vasculitis; Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Pauci Immune Vasculitis; Pauci-Immune Vasculitides; Pauci-Immune Vasculitis; Vasculitide, ANCA-Associated; Vasculitides, ANCA-Associated; Vasculitides, Pauci-Immune; Vasculitis, ANCA-Associated; Vasculitis, Pauci-Immune
SNOMED CT: Antineutrophil cytoplasmic antibody positive vasculitis (722191003); Antineutrophil cytoplasmic antibody (ANCA) positive vasculitis (722191003)
 
Monarch Initiative: MONDO:0015492
Orphanet: ORPHA156152

Definition

Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnti-neutrophil cytoplasmic antibody-associated vasculitis
Follow this link to review classifications for Anti-neutrophil cytoplasmic antibody-associated vasculitis in Orphanet.

Professional guidelines

PubMed

Sada KE, Kaname S, Higuchi T, Furuta S, Nagasaka K, Nanki T, Tsuboi N, Amano K, Dobashi H, Hiromura K, Bando M, Wada T, Arimura Y, Makino H, Harigai M
Mod Rheumatol 2023 Dec 22;34(1):144-150. doi: 10.1093/mr/road017. PMID: 36715080
King C, Harper L, Little M
Best Pract Res Clin Rheumatol 2018 Feb;32(1):125-136. Epub 2018 Aug 28 doi: 10.1016/j.berh.2018.07.009. PMID: 30526892
Shi L
Rheumatol Int 2017 Nov;37(11):1779-1788. Epub 2017 Sep 27 doi: 10.1007/s00296-017-3818-y. PMID: 28956128

Recent clinical studies

Etiology

Yaseen K, Mandell BF
Postgrad Med 2023 Jan;135(sup1):3-13. Epub 2022 Jul 21 doi: 10.1080/00325481.2022.2102368. PMID: 35831990
Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, Kullman J, Lyons PA, Merkel PA, Savage COS, Specks U, Kain R
Nat Rev Dis Primers 2020 Aug 27;6(1):71. doi: 10.1038/s41572-020-0204-y. PMID: 32855422
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
Yates M, Watts R
Clin Med (Lond) 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60. PMID: 28148583Free PMC Article
Sharma A, Dogra S, Sharma K
Dermatol Clin 2015 Jul;33(3):475-87. doi: 10.1016/j.det.2015.03.012. PMID: 26143427

Diagnosis

Arnold S, Kitching AR, Witko-Sarsat V, Wiech T, Specks U, Klapa S, Comdühr S, Stähle A, Müller A, Lamprecht P
Lancet Rheumatol 2024 May;6(5):e300-e313. Epub 2024 Apr 1 doi: 10.1016/S2665-9913(24)00025-0. PMID: 38574743
Chalkia A, Jayne D
Nephrol Dial Transplant 2024 May 31;39(6):944-955. doi: 10.1093/ndt/gfad237. PMID: 37947275Free PMC Article
Hunter RW, Welsh N, Farrah TE, Gallacher PJ, Dhaun N
BMJ 2020 Apr 14;369:m1070. doi: 10.1136/bmj.m1070. PMID: 32291255Free PMC Article
Salvador F
Eur J Intern Med 2020 Apr;74:18-28. Epub 2020 Jan 29 doi: 10.1016/j.ejim.2020.01.011. PMID: 32005600
Yates M, Watts R
Clin Med (Lond) 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60. PMID: 28148583Free PMC Article

Therapy

Chalkia A, Jayne D
Nephrol Dial Transplant 2024 May 31;39(6):944-955. doi: 10.1093/ndt/gfad237. PMID: 37947275Free PMC Article
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1088-1105. Epub 2021 Jul 8 doi: 10.1002/acr.24634. PMID: 34235880
Jayne DRW, Merkel PA, Schall TJ, Bekker P; ADVOCATE Study Group
N Engl J Med 2021 Feb 18;384(7):599-609. doi: 10.1056/NEJMoa2023386. PMID: 33596356
Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, Hawley CM, Khalidi N, Floßmann O, Wald R, Girard LP, Levin A, Gregorini G, Harper L, Clark WF, Pagnoux C, Specks U, Smyth L, Tesar V, Ito-Ihara T, de Zoysa JR, Szczeklik W, Flores-Suárez LF, Carette S, Guillevin L, Pusey CD, Casian AL, Brezina B, Mazzetti A, McAlear CA, Broadhurst E, Reidlinger D, Mehta S, Ives N, Jayne DRW; PEXIVAS Investigators
N Engl J Med 2020 Feb 13;382(7):622-631. doi: 10.1056/NEJMoa1803537. PMID: 32053298Free PMC Article
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

Sharma P, Zonozi R, Geetha D
Adv Kidney Dis Health 2024 May;31(3):194-205. doi: 10.1053/j.akdh.2024.04.005. PMID: 39004459
Chalkia A, Jayne D
Nephrol Dial Transplant 2024 May 31;39(6):944-955. doi: 10.1093/ndt/gfad237. PMID: 37947275Free PMC Article
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P, Shin JI
Int J Mol Sci 2020 Oct 3;21(19) doi: 10.3390/ijms21197319. PMID: 33023023Free PMC Article
Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, Hawley CM, Khalidi N, Floßmann O, Wald R, Girard LP, Levin A, Gregorini G, Harper L, Clark WF, Pagnoux C, Specks U, Smyth L, Tesar V, Ito-Ihara T, de Zoysa JR, Szczeklik W, Flores-Suárez LF, Carette S, Guillevin L, Pusey CD, Casian AL, Brezina B, Mazzetti A, McAlear CA, Broadhurst E, Reidlinger D, Mehta S, Ives N, Jayne DRW; PEXIVAS Investigators
N Engl J Med 2020 Feb 13;382(7):622-631. doi: 10.1056/NEJMoa1803537. PMID: 32053298Free PMC Article
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

Clinical prediction guides

Benichou N, Charles P, Terrier B, Jones RB, Hiemstra T, Mouthon L, Bajema I, Berden A, Thervet E, Guillevin L, Jayne D, Karras A; French Vasculitis Study Group (FVSG) and European Vasculitis Society (EUVAS) investigators
Kidney Int 2023 Jun;103(6):1144-1155. Epub 2023 Mar 20 doi: 10.1016/j.kint.2023.02.029. PMID: 36940799
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
Perez Alamino R, Martínez C, Espinoza LR
Curr Rheumatol Rep 2013 Aug;15(8):348. doi: 10.1007/s11926-013-0348-9. PMID: 23794082
Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, Flossmann O, Hall C, Hollywood J, Jayne D, Jones R, Lanyon P, Muir A, Scott D, Young L, Luqmani RA
Ann Rheum Dis 2009 Dec;68(12):1827-32. Epub 2008 Dec 3 doi: 10.1136/ard.2008.101279. PMID: 19054820

Recent systematic reviews

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
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
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1088-1105. Epub 2021 Jul 8 doi: 10.1002/acr.24634. PMID: 34235880
Guchelaar NAD, Waling MM, Adhin AA, van Daele PLA, Schreurs MWJ, Rombach SM
Autoimmun Rev 2021 Jan;20(1):102716. Epub 2020 Nov 13 doi: 10.1016/j.autrev.2020.102716. PMID: 33197574

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