HLAVNÍ TÉMA ANCA-asociované vaskulitidy – diagnostické a léčebné možnosti | 413 / Vnitř Lék. 2025;71(7):408-413 / VNITŘNÍ LÉKAŘSTVÍ www.casopisvnitrnilekarstvi.cz cílenou na IL-5, kterou se pak doporučuje využít také v léčbě relabující a refrakterní EGPA. Závěr ANCA-asociované vaskulitidy jsou vzácná onemocnění, na něž je vhodné pomýšlet zejména u pacientů s celkovými nespecifickými příznaky, nejasnou elevací zánětlivých parametrů a známkami postižení více orgánů. Typické jsou chronické rhinosinusitidy, nejasné plicní noduly či infiltráty, krvácení do plic a rychle progredující glomerulonefritida. Diagnóza může být potvrzena pozitivitou ANCA protilátek a/nebo provedením biopsie. V léčbě se uplatňuje kombinovaná imunosupresivní a biologická terapie (kortikosteroidy, cyklofosfamid, rituximab), nutná je individualizace léčebného postupu. Pro vzácnost onemocnění by pacienti měli být sledování v expertních centrech s dostupností multidisciplinárního týmu. PROHLÁŠENÍ AUTORŮ: Prohlášení o původnosti: Publikace byla zpracována s využitím uvedené literatury a nebyla publikována ani zaslána k recenznímu řízení do jiného média. Střet zájmů: ZH – platba za konzultace/přednášky: CSL Vifor, Astra Zeneca, GSK. VT – CSL Vifor, AstraZeneca, Boehringer Ingelheim, Novartis, Otsuka, Stada, Travere, Vera. Financování: Žádné. Poděkování: N/A. Registrace v databázích: N/A. Projednání etickou komisí: N/A. LITERATURA 1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. 2. Redondo-Rodriguez R, Mena-Vázquez N, Cabezas-Lucena AM, et al. Systematic Review and Metaanalysis of Worldwide Incidence and Prevalence of Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. J Clin Med. 2022;11(9):2573. 3. Rathmann J, Segelmark M, Englund M, et al. Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study. RMD Open. 2023;9:e002949. 4. Almaani S, Fussner LA, Brodsky S, et al. ANCA-Associated Vasculitis: An Update. J Clin Med. 2021;10(7):1446. 5. Sánchez Álamo B, Moi L, Bajema I, et al. Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2023;38(7):1655-1665. 6. Robson J, Doll H, Suppiah R, Flossmann O, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis. 2015;74(1):177-84. 7. Sanchez-Alamo B, Moi L, Bajema I, et al. Long-term outcome of kidney function in patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2024;39(9):1483-1493. 8. Ooi LJ, Hollick RJ, Brix SR. Challenges and advances in the management of antineutrophil cytoplasmic antibody vasculitis in 2025. Curr Opin Nephrol Hypertens. 2025;34(5):375-380. 9. Gopaluni S, Flossmann O, Little MA, et al. Effect of Disease Activity at Three and Six Months After Diagnosis on Long-Term Outcomes in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2019;71(5):784-791. 10. Hollick RJ, James WRG, Nicoll A, et al. Identifying key health system components associated with improved outcomes to inform the re-configuration of services for adults with rare autoimmune rheumatic diseases: a mixed-methods study. Lancet Rheumatol. 2024;6(6):e361-e373. 11. Bossuyt X, Cohen Tervaert JW, Arimura Y, et al. Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. 12. Hunter RW, Welsh N, Farrah TE, et al. ANCA associated vasculitis. BMJ. 2020;369:m1070. 13. Chevet B, Cornec D, Casal Moura M, et al. Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice. Rheumatology (Oxford). 2023;62(5):1787-1803. 14. McAdoo SP, Tanna A, Hrušková Z, et al. Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney Int. 2017;92(3):693-702. 15. McCarthy E, Mustafa M, Watts M. ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis. Eur J Case Rep Intern Med. 2017;4(8):000625. 16. Kidney Disease: Improving Global Outcomes (KDIGO) ANCA Vasculitis Work Group. KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis. Kidney Int. 2024;105(3S):S71-S116. 17. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83(1):30-47. 18. Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315-320. 19. Suppiah R, Robson JC, Grayson PC, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3):321-326. 20. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022;81(3):309-314. 21. Lyons PA, Rayner TF, Trivedi S, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med. 2012;367(3):214-23. 22. Hilhorst M, van Paassen P, Tervaert JW; Limburg Renal Registry. Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis. J Am Soc Nephrol. 2015;26(10):2314-27. 23. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36-44. 24. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670-80. 25. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-94. 26. van Daalen EE, Rizzo R, Kronbichler A, et al. Effect of rituximab on malignancy risk in patients with ANCA-associated vasculitis. Ann Rheum Dis. 2017;76(6):1064-1069. 27. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA- -associated vasculitis. N Engl J Med. 2010;363(3):221-32. 28. Jones RB, Tervaert JW, Hauser T, et a. Rituximab versus cyclophosphamide in ANCA- -associated renal vasculitis. N Engl J Med. 2010;363(3):211-20. 29. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371(19):1771-80. 30. Smith RM, Jones RB, Specks U, et al. Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: an international randomised controlled trial. Ann Rheum Dis. 2023;82(7):937-944. 31. Podestà MA, Mescia F, Ricchiuto A, et al. Predictors of hypogammaglobulinemia in ANCA-associated vasculitis after a rituximab-based induction: a multicentre study. Rheumatology (Oxford). 2023;62(8):2850-2854. 32. McAdoo SP, Pepper RJ, Prendecki M, et al. Long-Term Follow-Up of a Glucocorticoid Minimizing Regimen for the Treatment of Severe Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis. Kidney Int Rep. 2025;10(6):1874-1885. 33. Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. N Engl J Med. 2020;382(7):622-631. 34. Jayne DRW, Merkel PA, Schall TJ, et al. Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med. 2021;384(7):599-609. 35. Geetha D, Cortazar FB, Bruchfeld A, et al. Treatment With Avacopan in ANCA-Associated Vasculitis With Kidney Involvement. Kidney Int Rep. 2025;10(8):2751-2765. 36. Walsh M, Collister D, Zeng L, et al. The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis. BMJ. 2022;376:e064604. 37. Lyons PA, Peters JE, Alberici F, et al. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status. Nat Commun. 2019;10(1):5120. 38. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921-1932. 39. Wechsler ME, Nair P, Terrier B, et al. Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2024;390(10):911-921. 40. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73(8):1366-1383. 41. Biddle K, Jade J, Wilson-Morkeh H, et al. The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis. Rheumatology (Oxford). 2025;64(8):4470-4494. 42. Suppiah R, Mukhtyar C, Flossmann O, et al. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis. Rheumatology (Oxford). 2011;50(5):899-905. 43. Suppiah R, Flossman O, Mukhtyar C, et al. Measurement of damage in systemic vasculitis: a comparison of the Vasculitis Damage Index with the Combined Damage Assessment Index. Ann Rheum Dis. 2011;70(1):80-5. 44. Robson JC, Dawson J, Doll H, et al. Validation of the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire. Ann Rheum Dis. 2018;77(8):1157-1164.
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