rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2021-684-692rsp-3102Research ArticleМЕЖДУНАРОДНЫЕ И РОССИЙСКИЕ РЕКОМЕНДАЦИИ ПО ЛЕЧЕНИЮ РЕВМАТИЧЕСКИХ ЗАБОЛЕВАНИЙINTERNATIONAL AND RUSSIAN GUIDELINES FOR THE TREATMENT OF RHEUMATIC DISEASESОбзор рекомендаций по лечению АНЦА-ассоциированных системных васкулитов, представленных в 2021 г. Американской коллегией ревматологов и Фондом васкулитовReview of guideline for the management of ANCA-associated vasculitis, presented in 2021 by the American College of Rheumatology/Vasculitis Foundationhttps://orcid.org/0000-0003-2641-9785БекетоваТ. В.BeketovaT. V.

Бекетова Татьяна Валентиновна.

115522, Москва, Каширское шоссе, 34а; 121359, Москва, ул. Маршала Тимошенко, 15.

Tatiana V. Beketova.

115522, Moscow, Kashirskoye Highway, 34A; 121359, Moscow, Marshala Timoshenko str., 15.

tvbek@rambler.ruhttps://orcid.org/0000-0002-6373-332XПоповИ. Ю.PopovI. Yu.

115522, Москва, Каширское шоссе, 34а.

Ivan Yu. Popov.

115522, Moscow, Kashirskoye Highway, 34A.

https://orcid.org/0000-0001-8020-2494БабакВ. В.BabakV. V.

115522, Москва, Каширское шоссе, 34а.

Valeriya V. Babak.

115522, Moscow, Kashirskoye Highway, 34A.

Научно-исследовательский институт ревматологии им. В.А. Насоновой; Центральная клиническая больница с поликлиникой Управления делами Президента РФРоссияV.A. Nasonova Research Institute of Rheumatology; Central Clinical Hospital of the Administrative Directorate of the President of the Russian FederationRussian FederationНаучно-исследовательский институт ревматологии им. В.А. НасоновойРоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation202127122021596684692Copyright © Бекетова Т.В., Попов И.Ю., Бабак В.В., 20212021Бекетова Т.В., Попов И.Ю., Бабак В.В.Beketova T.V., Popov I.Y., Babak V.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/3102

Системные васкулиты (СВ), ассоциированные с антинейтрофильными цитоплазматическими антителами (АНЦА), относятся к тяжелым жизнеугрожающим аутоиммунным заболеваниям и остаются одной из важнейших проблем практической ревматологии. В статье обсуждаются новые рекомендации Американской коллегии ревматологов и Фонда васкулитов по лечению пациентов с АНЦА-СВ, включая гранулематоз с полиангиитом (ГПА), микроскопический полиангиит (МПА) и эозинофильный гранулематоз с полиангиитом (ЭГПА), которые были опубликованы в августе 2021 г. Они включают 26 рекомендаций и 5 дополнений для ГПА/МПА, а также 15 рекомендаций и 5 дополнений для ЭГПА, которые стали основанием для предложенных алгоритмов лечения пациентов с ГПА/МПА и ЭГПА. Особое внимание уделяется применению генно-инженерных биологических препаратов.

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of severe life-threatening autoimmune diseases, and one of the most important problems in practical rheumatology. This article discusses 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of AAVs, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The guideline features 26 recommendations and 5 upgraded position statements for GPA/ MPA, as well as 15 recommendations and 5 upgraded position statements for EGPA, which became the basis for the proposed algorithms for the treatment of patients with GPA/MPA and EGPA. Particular attention has been paid to the use of biologics.

системный васкулитантинейтрофильные цитоплазматические антителагранулематоз с полиангиитоммикроскопический полиангиитэозинофильный гранулематоз с полиангиитомритуксимабsystemic vasculitisantineutrophil cytoplasmic antibodiesgranulomatosis with polyangiitis (Wegener's)microscopic polyangiitiseosinophilic granulomatosis with polyangiitisrituximabReferencesChung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, 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. doi: 10.1002/art.41773Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, 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. doi: 10.1002/art.41773Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: Assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: Assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al.; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. doi: 10.1056/NEJMoa0909905Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al.; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. doi: 10.1056/NEJMoa0909905Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al.; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211-220. doi: 10.1056/NEJMoa0909169Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al.; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211-220. doi: 10.1056/NEJMoa0909169Kahn JE, Grandpeix-Guyodo C, Marroun I, Catherinot E, Mellot F, Roufosse F, et al. Sustained response to mepolizumab in refractory Churg - Strauss syndrome. J Allergy Clin Immunol. 2010;125(1):267-270. doi: 10.1016/j.jaci.2009.10.014Kahn JE, Grandpeix-Guyodo C, Marroun I, Catherinot E, Mellot F, Roufosse F, et al. Sustained response to mepolizumab in refractory Churg - Strauss syndrome. J Allergy Clin Immunol. 2010;125(1):267-270. doi: 10.1016/j.jaci.2009.10.014Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017;376(20):1921-1932. doi: 10.1056/NEJMoa1702079Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017;376(20):1921-1932. doi: 10.1056/NEJMoa1702079Menditto VG, Rossetti G, Olivari D, Angeletti A, Rocchi M, Gabrielli A, et al. Rituximab for eosinophilic granulomatosis with polyangiitis: A systematic review of observational studies. Rheumatol. 2021;60:1640-1650. doi: 10.1093/rheumatology/keab046Menditto VG, Rossetti G, Olivari D, Angeletti A, Rocchi M, Gabrielli A, et al. Rituximab for eosinophilic granulomatosis with polyangiitis: A systematic review of observational studies. Rheumatol. 2021;60:1640-1650. doi: 10.1093/rheumatology/keab046Akiyama M, Kaneko Y, Takeuchi T. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis: A systematic literature review. Autoimmun Rev. 2021;20:102737. doi: 10.1016/j.autrev.2020.102737Akiyama M, Kaneko Y, Takeuchi T. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis: A systematic literature review. Autoimmun Rev. 2021;20:102737. doi: 10.1016/j.autrev.2020.102737Walsh M, Merkel PA, Peh CA, Szpirt WM, Putehal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382:622-631. doi: 10.1056/NEJMoa1803537Walsh M, Merkel PA, Peh CA, Szpirt WM, Putehal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382:622-631. doi: 10.1056/NEJMoa1803537Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007;18:2180-2188. doi: 10.1681/ASN.2007010090Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007;18:2180-2188. doi: 10.1681/ASN.2007010090Walsh M, Casian A, Flossmann O, Westman K, Hoglund P, Pusey C, et al.; European Vasculitis Study Group (EUVAS). Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 2013;84(2):397-402. doi: 10.1038/ki.2013.131Walsh M, Casian A, Flossmann O, Westman K, Hoglund P, Pusey C, et al.; European Vasculitis Study Group (EUVAS). Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 2013;84(2):397-402. doi: 10.1038/ki.2013.131Teixeira V, Mohammad AJ, Jones RB, Smith R, Jayne D. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open. 2019;5(1):e000905. doi: 10.1136/rmdopen-2019-000905Teixeira V, Mohammad AJ, Jones RB, Smith R, Jayne D. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open. 2019;5(1):e000905. doi: 10.1136/rmdopen-2019-000905Crickx E, Machelart I, Lazaro E, Kahn JE, Cohen-Aubart F, Martin T, et al. Intravenous immunoglobulin as an immunomodulating agent in antineutrophil cytoplasmic antibody-associated vas-culitides: A French nationwide study of ninety-two patients. Arthritis Rheumatol. 2016;68:702-712. doi: 10.1002/art.39472Crickx E, Machelart I, Lazaro E, Kahn JE, Cohen-Aubart F, Martin T, et al. Intravenous immunoglobulin as an immunomodulating agent in antineutrophil cytoplasmic antibody-associated vas-culitides: A French nationwide study of ninety-two patients. Arthritis Rheumatol. 2016;68:702-712. doi: 10.1002/art.39472De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461-2469. doi: 10.1002/art.21142De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461-2469. doi: 10.1002/art.21142Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaitre O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771-1780. doi: 10.1056/NEJMoa1404231Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaitre O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771-1780. doi: 10.1056/NEJMoa1404231Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790-2803. doi: 10.1056/NEJMoa0802311Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790-2803. doi: 10.1056/NEJMoa0802311Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: A randomized controlled trial. JAMA. 2010;304:2381-2388. doi: 10.1001/jama.2010.1658Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: A randomized controlled trial. JAMA. 2010;304:2381-2388. doi: 10.1001/jama.2010.1658Puéchal X, Pagnoux C, Baron G, Quémeneur T, Néel A, Agard C, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg - Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: A randomized, controlled trial. Arthritis Rheumatol. 2017;69:2175-2186. doi: 10.1002/art.40205Puéchal X, Pagnoux C, Baron G, Quémeneur T, Néel A, Agard C, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg - Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: A randomized, controlled trial. Arthritis Rheumatol. 2017;69:2175-2186. doi: 10.1002/art.40205Metzler C, Miehle N, Manger K, Iking-Konert C, de Groot K, Hellmich B, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford). 2007;46:1087-1091. doi: 10.1093/rheumatology/kem029Metzler C, Miehle N, Manger K, Iking-Konert C, de Groot K, Hellmich B, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford). 2007;46:1087-1091. doi: 10.1093/rheumatology/kem029Kado R, Sanders G, McCune WJ. Diagnostic and therapeutic considerations in patients with hypogammaglobulinemia after rituximab therapy (review). Curr Opin Rheumatol. 2017;29:228-233. doi: 10.1097/BOR.0000000000000377Kado R, Sanders G, McCune WJ. Diagnostic and therapeutic considerations in patients with hypogammaglobulinemia after rituximab therapy (review). Curr Opin Rheumatol. 2017;29:228-233. doi: 10.1097/BOR.0000000000000377Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, et al. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014;371:1198-1207. doi: 10.1056/NEJMoa1403290Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, et al. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014;371:1198-1207. doi: 10.1056/NEJMoa1403290Joshi L, Tanna A, McAdoo SP, Medjeral-Thomas N, Taylor SR, Sandhu G, et al. Long-term outcomes of rituximab therapy in ocular granulomatosis with polyangiitis: Impact on localized and nonlocalized disease. Ophthalmology. 2015;122:1262-1268. doi: 10.1016/j.ophtha.2015.01.016Joshi L, Tanna A, McAdoo SP, Medjeral-Thomas N, Taylor SR, Sandhu G, et al. Long-term outcomes of rituximab therapy in ocular granulomatosis with polyangiitis: Impact on localized and nonlocalized disease. Ophthalmology. 2015;122:1262-1268. doi: 10.1016/j.ophtha.2015.01.016Durel CA, Hot A, Trefond L, Aumaitre O, Pugnet G, Samson M, et al. Orbital mass in ANCA-associated vasculitides: Data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients. Rheumatology (Oxford). 2019;58:1565-1573. doi: 10.1093/rheumatology/Kez071Durel CA, Hot A, Trefond L, Aumaitre O, Pugnet G, Samson M, et al. Orbital mass in ANCA-associated vasculitides: Data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients. Rheumatology (Oxford). 2019;58:1565-1573. doi: 10.1093/rheumatology/Kez071Girard C, Charles P, Terrier B, Bussonne G, Cohen P, Pagnoux C, et al. Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener's): A report on 26 cases. Medicine (Baltimore). 2015;94:e1088. doi: 10.1097/MD.0000000000001088Girard C, Charles P, Terrier B, Bussonne G, Cohen P, Pagnoux C, et al. Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener's): A report on 26 cases. Medicine (Baltimore). 2015;94:e1088. doi: 10.1097/MD.0000000000001088Sagmeister MS, Grigorescu M, Schonermarck U. Kidney transplantation in ANCA-associated vasculitis. J Nephrol. 2019;32:919-926. doi: 10.1007/s40620-019-00642-xSagmeister MS, Grigorescu M, Schonermarck U. Kidney transplantation in ANCA-associated vasculitis. J Nephrol. 2019;32:919-926. doi: 10.1007/s40620-019-00642-xGeetha D, Eirin A, True K, Valentina Irazabal M, Specks U, Seo P, et al. Renal transplantation in antineutrophil cytoplasmic antibody-associated vasculitis: A multicenter experience. Transplantation. 2011;91:1370-1375. doi: 10.1097/TP.0b013e31821ab9aaGeetha D, Eirin A, True K, Valentina Irazabal M, Specks U, Seo P, et al. Renal transplantation in antineutrophil cytoplasmic antibody-associated vasculitis: A multicenter experience. Transplantation. 2011;91:1370-1375. doi: 10.1097/TP.0b013e31821ab9aaSachse F, Stoll W. Nasal surgery in patients with systemic disorders. GMS Curr Top Otorhinolaryngol Head Neck Surg. 2010;9:Doc02. doi: 10.3205/cto000066Sachse F, Stoll W. Nasal surgery in patients with systemic disorders. GMS Curr Top Otorhinolaryngol Head Neck Surg. 2010;9:Doc02. doi: 10.3205/cto000066Congdon D, Sherris DA, Specks U, McDonald T. Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis. Laryngoscope. 2002;112:731-737. doi: 10.1097/00005537-200204000-00025Congdon D, Sherris DA, Specks U, McDonald T. Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis. Laryngoscope. 2002;112:731-737. doi: 10.1097/00005537-200204000-00025Tomasson G, Grayson PC, Mahr AD, Lavalley M, Merkel PA. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis - A meta-analysis. Rheumatology (Oxford). 2012;51(1):100-109. doi: 10.1093/rheumatology/ker280Tomasson G, Grayson PC, Mahr AD, Lavalley M, Merkel PA. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis - A meta-analysis. Rheumatology (Oxford). 2012;51(1):100-109. doi: 10.1093/rheumatology/ker280Charles P, Terrier B, Perrodeau E, Cohen P, Faguer S, Huart A, et al. Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: Results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2). Ann Rheum Dis. 2018;77:1143-1149. doi: 10.1136/annrheumdis-2017-212878Charles P, Terrier B, Perrodeau E, Cohen P, Faguer S, Huart A, et al. Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: Results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2). Ann Rheum Dis. 2018;77:1143-1149. doi: 10.1136/annrheumdis-2017-212878

The authors declare that there are no conflicts of interest present.