ANCA-associated Vasculitis

Overview

Journal Clin Med (Lond)

Specialty General Medicine

Date 2017 Feb 3

PMID 28148583

Citations 90

Authors

Max Yates

Richard Watts

Affiliations

Soon will be listed here.

Abstract

The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome). This review examines recent developments in the pathogenesis and treatment of AAV, focusing on developments in treatment following the introduction of rituximab, in particular.

Citing Articles

Azathioprine Hypersensitivity Syndrome Mimicking Herpes Zoster and Linear IgA Dermatosis Presentation: A Case Report.

Budair F, Alfaraj M, Alharthi K, Altabash M, Alratroot J Acta Derm Venereol. 2025; 105:adv42536.

PMID: 39964214 PMC: 11848941. DOI: 10.2340/actadv.v105.42536.

Comparison of the clinicopathological characteristics of children with anti-neutrophil cytoplasmic antibody-associated vasculitis with/without infection at diagnosis.

Long L, Tan M, Deng H, Tang H, Lin X, Zhang M BMC Nephrol. 2025; 26(1):7.

PMID: 39762790 PMC: 11706063. DOI: 10.1186/s12882-024-03919-x.

Rare Clinical Manifestation of Vasculitis.

Manole O, Haba M, Matei I, Onofrei V Diagnostics (Basel). 2024; 14(23).

PMID: 39682532 PMC: 11640520. DOI: 10.3390/diagnostics14232623.

Case report: Granulomatosis with polyangiitis patient presented with a mass in the aortic root.

Teng Y, Chen M, Chen Z, Zhang X, Li Z, Liao S Front Immunol. 2024; 15:1373769.

PMID: 39669581 PMC: 11635199. DOI: 10.3389/fimmu.2024.1373769.

Renal-Limited Presentation of Hydralazine-Induced Anti-neutrophil Cytoplasmic Antibody Vasculitis: A Case Report.

Lurie A, Guntupalli L, Finnigan N, Ahmed U Cureus. 2024; 16(9):e69330.

PMID: 39398788 PMC: 11471049. DOI: 10.7759/cureus.69330.

References

Jayne D, Rasmussen N, Andrassy K, Bacon P, Cohen Tervaert J, Dadoniene J . A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003; 349(1):36-44. DOI: 10.1056/NEJMoa020286. View

Groot K, Rasmussen N, Bacon P, Cohen Tervaert J, Feighery C, Gregorini G . Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005; 52(8):2461-9. DOI: 10.1002/art.21142. View

Hellmich B, Flossmann O, Gross W, Bacon P, Cohen-Tervaert J, Guillevin L . EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2006; 66(5):605-17. PMC: 2703775. DOI: 10.1136/ard.2006.062711. View

Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S . Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2008; 68(12):1827-32. DOI: 10.1136/ard.2008.101279. View

Jones R, Ferraro A, Chaudhry A, Brogan P, Salama A, Smith K . A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2009; 60(7):2156-68. DOI: 10.1002/art.24637. View

Jones R, Cohen Tervaert J, Hauser T, Luqmani R, Morgan M, Peh C . Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010; 363(3):211-20. DOI: 10.1056/NEJMoa0909169. View

Stone J, Merkel P, Spiera R, Seo P, Langford C, Hoffman G . Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; 363(3):221-32. PMC: 3137658. DOI: 10.1056/NEJMoa0909905. View

Harper L, Morgan M, Walsh M, Hoglund P, Westman K, Flossmann O . Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2011; 71(6):955-60. DOI: 10.1136/annrheumdis-2011-200477. View

Smith R, Jones R, Guerry M, Laurino S, Catapano F, Chaudhry A . Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012; 64(11):3760-9. DOI: 10.1002/art.34583. View

10.

Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F . 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2012; 65(1):1-11. DOI: 10.1002/art.37715. View

11.

Robson J, Doll H, Suppiah R, Flossmann O, Harper L, Hoglund P . Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis. 2013; 74(1):177-84. DOI: 10.1136/annrheumdis-2013-203927. View

12.

Mooney J, Spalding N, Poland F, Grayson P, Leduc R, McAlear C . The informational needs of patients with ANCA-associated vasculitis-development of an informational needs questionnaire. Rheumatology (Oxford). 2014; 53(8):1414-21. PMC: 4103516. DOI: 10.1093/rheumatology/keu026. View

13.

Ntatsaki E, Carruthers D, Chakravarty K, DCruz D, Harper L, Jayne D . BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014; 53(12):2306-9. DOI: 10.1093/rheumatology/ket445. View

14.

Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaitre O, Cohen P . Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014; 371(19):1771-80. DOI: 10.1056/NEJMoa1404231. View

15.

Watts R, Mahr A, Mohammad A, Gatenby P, Basu N, Flores-Suarez L . Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Nephrol Dial Transplant. 2015; 30 Suppl 1:i14-22. DOI: 10.1093/ndt/gfv022. View

16.

Hilhorst M, van Paassen P, Cohen Tervaert J . Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis. J Am Soc Nephrol. 2015; 26(10):2314-27. PMC: 4587702. DOI: 10.1681/ASN.2014090903. View

17.

Unizony S, Villarreal M, Miloslavsky E, Lu N, Merkel P, Spiera R . Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis. 2015; 75(6):1166-9. PMC: 4908815. DOI: 10.1136/annrheumdis-2015-208073. View

18.

Koh J, Kemna M, Cohen Tervaert J, Kim W . Editorial: Can an Increase in Antineutrophil Cytoplasmic Autoantibody Titer Predict Relapses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis?. Arthritis Rheumatol. 2016; 68(7):1571-3. DOI: 10.1002/art.39639. View

19.

Yates M, Watts R, Bajema I, Cid M, Crestani B, Hauser T . EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016; 75(9):1583-94. DOI: 10.1136/annrheumdis-2016-209133. View

20.

Davies D, Moran J, Niall J, RYAN G . Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. Br Med J (Clin Res Ed). 1982; 285(6342):606. PMC: 1499415. DOI: 10.1136/bmj.285.6342.606. View