J Rheum Dis.  2023 Apr;30(2):72-87. 10.4078/jrd.2022.0002.

Management of antineutrophil cytoplasmic antibody-associated vasculitis: a review of recent guidelines

Affiliations
  • 1Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea
  • 2Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
  • 3Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune rheumatic disease consisting of three discrete diagnoses of microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. Among diseases treated in a rheumatology department, AAV has poor clinical outcomes, with high rates of mortality and progression to end-stage renal disease and frequent disease relapse. Due to the frequent negative patient outcomes, optimal therapeutic strategies are essential in the management of AAV. In the present review, four guidelines for management of AAV are summarized: British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV; European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendation for the management of AAV; 2021 American College of Rheumatology (ACR)/Vasculitis Foundation Guideline for the Management of AAV; Kidney Disease: Improving Global Outcome (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases, which will aid in clinicians’ medical decisions. Finally, the summary of the 2022 Update of the EULAR Recommendations on the Management of AAV, presented in the EULAR Congress 2022 is also introduced.

Keyword

Antineutrophil cytoplasmic antibody; Vasculitis; Treatment; Guideline

Cited by  1 articles

Renal prognosis in patients with new-onset microscopic polyangiitis and granulomatosis with polyangiitis requiring dialysis and potential predictor of renal function recovery
Sung Soo Ahn
J Rheum Dis. 2024;31(1):1-2.    doi: 10.4078/jrd.2023.0079.


Reference

1. Hunter RW, Welsh N, Farrah TE, Gallacher PJ, Dhaun N. 2020; ANCA associated vasculitis. BMJ. 369:m1070. DOI: 10.1136/bmj.m1070. PMID: 32291255. PMCID: PMC7179255.
Article
2. Ahn SS, Park YB, Lee SW. 2021; Serological biomarkers and indices for the current activity and prognosis of ANCA-associated vasculitis: experience in a single centre in Korea. Yonsei Med J. 62:279–87. DOI: 10.3349/ymj.2021.62.4.279. PMID: 33779081. PMCID: PMC8007433.
Article
3. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2013; 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 65:1–11. DOI: 10.1002/art.37715. PMID: 23045170.
4. Westman K, Flossmann O, Gregorini G. 2015; The long-term outcomes of systemic vasculitis. Nephrol Dial Transplant. 30 Suppl 1:i60–6. DOI: 10.1093/ndt/gfu392. PMID: 25601266.
Article
5. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, et al. 2011; Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 70:488–94. DOI: 10.1136/ard.2010.137778. PMID: 21109517.
Article
6. Gopaluni S, Flossmann O, Little MA, O'Hara P, Bekker P, Jayne D. 2019; Effect of disease activity at three and six months after diagnosis on long-term outcomes in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 71:784–91. DOI: 10.1002/art.40776. PMID: 30418705.
Article
7. Rhee RL, Hogan SL, Poulton CJ, McGregor JA, Landis JR, Falk RJ, et al. 2016; Trends in long-term outcomes among patients with antineutrophil cytoplasmic antibody-associated vasculitis with renal disease. Arthritis Rheumatol. 68:1711–20. DOI: 10.1002/art.39614. PMID: 26814428. PMCID: PMC4920688.
Article
8. Watanabe H, Sada KE, Harigai M, Amano K, Dobashi H, Takasaki Y, et al. 2021; Exploratory classification of clinical phenotypes in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis using cluster analysis. Sci Rep. 11:5223. DOI: 10.1038/s41598-021-84627-6. PMID: 33664381. PMCID: PMC7933174.
Article
9. Ahn SS, Lim H, Lee CH, Park YB, Park JS, Lee SW. 2022; Secular trends of incidence, prevalence, and healthcare economic burden in ANCA-associated vasculitis: an analysis of the 2002-2018 South Korea National Health Insurance Database. Front Med (Lausanne). 9:902423. DOI: 10.3389/fmed.2022.902423. PMID: 35872769. PMCID: PMC9300883.
Article
10. Salama AD. 2020; Relapse in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis. Kidney Int Rep. 5:7–12. DOI: 10.1016/j.ekir.2019.10.005. PMID: 31922056. PMCID: PMC6943777.
11. White J, Dubey S. 2023; Eosinophilic granulomatosis with polyangiitis: a review. Autoimmun Rev. 22:103219. DOI: 10.1016/j.autrev.2022.103219. PMID: 36283646.
Article
12. Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, et al. 2007; EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 66:605–17. DOI: 10.1136/ard.2006.062711. PMID: 17170053. PMCID: PMC2703775.
13. WGET Research Group. 2002; Design of the Wegener's Granulomatosis Etanercept Trial (WGET). Control Clin Trials. 23:450–68. DOI: 10.1016/S0197-2456(02)00209-X. PMID: 12161090.
14. Mukhtyar C, Hellmich B, Jayne D, Flossmann O, Luqmani R. 2006; Remission in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Clin Exp Rheumatol. 24(6 Suppl 43):S–93.
15. Ntatsaki E, Carruthers D, Chakravarty K, D'Cruz D, Harper L, Jayne D, et al. 2014; BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 53:2306–9. DOI: 10.1093/rheumatology/ket445. PMID: 24729399.
Article
16. Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. 2009; Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 68:1827–32. DOI: 10.1136/ard.2008.101279. PMID: 19054820.
Article
17. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. 1994; Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 87:671–8.
18. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. 2016; EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 75:1583–94. DOI: 10.1136/annrheumdis-2016-209133. PMID: 27338776.
Article
19. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. 2009; EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 68:310–7. DOI: 10.1136/ard.2008.088096. PMID: 18413444.
Article
20. Furuta S, Iwamoto T, Nakajima H. 2019; Update on eosinophilic granulomatosis with polyangiitis. Allergol Int. 68:430–6. DOI: 10.1016/j.alit.2019.06.004. PMID: 31266709.
Article
21. Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021; 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 73:1366–83. DOI: 10.1002/art.41773. PMID: 34235894.
Article
22. Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021; 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res (Hoboken). 73:1088–105. DOI: 10.1002/acr.24634. PMID: 34235880.
Article
23. Deeks ED. 2016; Mepolizumab: a review in eosinophilic asthma. BioDrugs. 30:361–70. DOI: 10.1007/s40259-016-0182-5. PMID: 27311938.
Article
24. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. 1996; Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 75:17–28. DOI: 10.1097/00005792-199601000-00003. PMID: 8569467.
Article
25. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL. 2011; The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 90:19–27. DOI: 10.1097/MD.0b013e318205a4c6. PMID: 21200183.
26. Jayne DRW, Merkel PA, Schall TJ, Bekker P. 2021; Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med. 384:599–609. DOI: 10.1056/NEJMoa2023386. PMID: 33596356.
Article
27. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. 2021; KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 100(4S):S1–276.
28. Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, et al. 2020; Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 382:622–31. DOI: 10.1056/NEJMoa1803537. PMID: 32053298. PMCID: PMC7325726.
Article
29. Yoon T, Ahn SS, Pyo JY, Song JJ, Park YB, Lee SW. 2021; Serum clusterin level could reflect the current activity of antineutrophil cytoplasmic antibody-associated vasculitis. Yonsei Med J. 62:1016–22. DOI: 10.3349/ymj.2021.62.11.1016. PMID: 34672135. PMCID: PMC8542470.
Article
30. Watts RA, Hatemi G, Burns JC, Mohammad AJ. 2022; Global epidemiology of vasculitis. Nat Rev Rheumatol. 18:22–34. DOI: 10.1038/s41584-021-00718-8. PMID: 34853411. PMCID: PMC8633913.
Article
31. Yaseen K, Mandell BF. DOI: 10.1080/00325481.2022.2102368. PMID: 35831990.
32. Okayama Y, Matsumoto H, Odajima H, Takahagi S, Hide M, Okubo K. 2020; Roles of omalizumab in various allergic diseases. Allergol Int. 69:167–77. DOI: 10.1016/j.alit.2020.01.004. PMID: 32067933.
Article
33. Basta F, Mazzuca C, Nucera E, Schiavino D, Afeltra A, Antonelli Incalzi R. 2020; Omalizumab in eosinophilic granulomatosis with polyangiitis: friend or foe? A systematic literature review. Clin Exp Rheumatol. 38 Suppl 124(2):214–20.
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