rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2017-12-16rsp-2332Research ArticleМЕЖДУНАРОДНЫЕ И РОССИЙСКИЕ РЕКОМЕНДАЦИИ ПО ЛЕЧЕНИЮ РЕВМАТИЧЕСКИХ ЗАБОЛЕВАНИЙINTERNATIONAL AND RUSSIAN GUIDELINES FOR THE TREATMENT OF RHEUMATIC DISEASESЕвропейские (EULAR/ERA-EDTA) рекомендации по диагностике и лечению АНЦА-ассоциированных системных васкулитов – 2016THE 2016 EUROPEAN (EULAR/ERA-EDTA) RECOMMENDATIONS FOR THE DIAGNOSIS AND MANAGEMENT OF ANCA-ASSOCIATED SYSTEMIC VASCULITISБекетоваТ. В.BeketovaT. V.

Москва, Россия 115522 Москва, Каширское шоссе, 34А

Moscow, Russia 34A, Kashirskoe Shosse, Moscow 115522

TVBEKTVBEK@rambler.ruФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation2017080320175511216Copyright © Бекетова Т.В., 20172017Бекетова Т.В.Beketova T.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2332

В начале 2016 г. международной группой экспертов – членов Европейской антиревматической лиги (EULAR), Европейской ассоциации по изучению болезней почек (ERA-EDTA) и Европейского общества изучения васкулитов (EUVAS) были опубликованы новые рекомендации по диагностике и лечению системных васкулитов (СВ), ассоциированных с антинейтрофильными цитоплазматическими антителами (АНЦА). Эти рекомендации являются важным этапом в развитии концепции АНЦА-СВ и стали результатом систематизированного обобщения современных научных достижений и клинического опыта. Предложенные 15 рекомендаций EULAR/ERA-EDTA освещают широкий круг проблем АНЦА-СВ и должны способствовать совершенствованию диагностики, повышению эффективности терапии и улучшению прогноза этих заболеваний. Целью публикации является общая характеристика основных положений рекомендаций.

In early 2016, the international group of expert members of the European League Against Rheumatism (EULAR), the European Renal Association – European Dialysis and Transplant Association (ERA-EDTA), and the European Vasculitis Society (EUVAS) published new guidelines for the diagnosis and management of antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (SV). These guidelines are an important step in the development of the concept of ANCA-SV and have resulted from the systematic generalization of current scientific achievements and clinical experience. Fifteen recommendations proposed by the EULAR/ERA-EDTA highlight a wide range of problems of ANCA-SV and should contribute to the improvement of the diagnosis, efficiency of therapy and prognosis of these diseases. The aim of the publication is to provide the general characteristics of the main provisions of the guidelines.

системный васкулитантинейтрофильные цитоплазматические антителагранулематоз с полиангиитом (Вегенера)микроскопический полиангиитэозинофильный гранулематоз с полиангиитомритуксимаб.systemic vasculitisantineutrophil cytoplasmic antibodiesgranulomatosis with polyangiitis (Wegener's)microscopic polyangiitiseosinophilic granulomatosis with polyangiitisrituximabReferencesYates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Jun 23. pii: annrheumdis- 2016-209133. doi: 10.1136/annrheumdis-2016-209133Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Jun 23. pii: annrheumdis- 2016-209133. doi: 10.1136/annrheumdis-2016-209133Aasarod K, Bostad L, Hammerstrom J, et al. Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. Nephrol Dial Transplant. 2001;16:953-60. doi: 10.1093/ndt/16.5.953Aasarod K, Bostad L, Hammerstrom J, et al. Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. Nephrol Dial Transplant. 2001;16:953-60. doi: 10.1093/ndt/16.5.953Schnabel A, Holl-Ulrich K, Dalhoff K, et al. Efficacy of transbronchial biopsy in pulmonary vaculitides. Eur Respir J. 1997;10:2738-43. doi: 10.1183/09031936.97.10122738Schnabel A, Holl-Ulrich K, Dalhoff K, et al. Efficacy of transbronchial biopsy in pulmonary vaculitides. Eur Respir J. 1997;10:2738-43. doi: 10.1183/09031936.97.10122738Corapi KM, Chen JL, Balk EM, et al. Bleeding complications of native kidney biopsy: a systematic review and meta-analysis. Am J Kidney Dis. 2012;60:62-73. doi: 10.1053/j.ajkd.2012.02.330Corapi KM, Chen JL, Balk EM, et al. Bleeding complications of native kidney biopsy: a systematic review and meta-analysis. Am J Kidney Dis. 2012;60:62-73. doi: 10.1053/j.ajkd.2012.02.330De 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 rial. Ann Intern Med. 2009;150:670-80. doi: 10.7326/0003-4819-150-10-200905190-00004De 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 rial. Ann Intern Med. 2009;150:670-80. doi: 10.7326/0003-4819-150-10-200905190-00004Guillevin L, Cordier JF, Lhote F, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum. 1997;40:2187- 98. doi: 10.1002/1529-0131(199712)40:12<2187::AIDART12>3.0.CO;2-HGuillevin L, Cordier JF, Lhote F, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum. 1997;40:2187- 98. doi: 10.1002/1529-0131(199712)40:12<2187::AIDART12>3.0.CO;2-HHarper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for nduction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71:955-60. doi: 10.1136/annrheumdis-2011-200477Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for nduction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71:955-60. doi: 10.1136/annrheumdis-2011-200477Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA- associated renal vasculitis. N Engl J Med. 2010;363:211-20. doi: 10.1056/NEJMoa0909169Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA- associated renal vasculitis. N Engl J Med. 2010;363:211-20. doi: 10.1056/NEJMoa0909169Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA- associated vasculitis. N Engl J Med. 2010;363:221-32. doi: 10.1056/NEJMoa0909905Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA- associated vasculitis. N Engl J Med. 2010;363:221-32. doi: 10.1056/NEJMoa0909905De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461-9. doi: 10.1002/art.21142De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461-9. doi: 10.1002/art.21142Langford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000;43:1836-40. doi: 10.1002/1529- 0131(200008)43: 8<1836: :AIDANR20>3.0.CO;2-RLangford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000;43:1836-40. doi: 10.1002/1529- 0131(200008)43: 8<1836: :AIDANR20>3.0.CO;2-RMetzler C, Hellmich B, Gause A, et al. Churg Strauss syndrome – successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment. Clin Exp Rheumatol. 2004;22(6 Suppl.):S-52-61.Metzler C, Hellmich B, Gause A, et al. Churg Strauss syndrome – successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment. Clin Exp Rheumatol. 2004;22(6 Suppl.):S-52-61.Monach PA, Arnold LM, Merkel PA. Incidence and prevention of bladder toxicity from cyclophosphamide in the treatment of rheumatic diseases: A data-driven review. Arthritis Rheum. 2010;62:9-21. doi: 10.1002/art.25061Monach PA, Arnold LM, Merkel PA. Incidence and prevention of bladder toxicity from cyclophosphamide in the treatment of rheumatic diseases: A data-driven review. Arthritis Rheum. 2010;62:9-21. doi: 10.1002/art.25061Miloslavsky EM, Specks U, Merkel PA, et al. Outcomes of nonsevere relapses in antineutrophil cytoplasmic antibody-associated vasculitis treated with glucocorticoids. Arthritis Rheum. 2015;67:1629-36. doi: 10.1002/art.39104Miloslavsky EM, Specks U, Merkel PA, et al. Outcomes of nonsevere relapses in antineutrophil cytoplasmic antibody-associated vasculitis treated with glucocorticoids. Arthritis Rheum. 2015;67:1629-36. doi: 10.1002/art.39104Klemmer PJ, Chalermskulrat W, Reif MS, et al. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with Small-Vessel vasculitis. Am J Kidney Dis. 2003;42:1149-53.doi: 10.1053/j.ajkd.2003.08.015Klemmer PJ, Chalermskulrat W, Reif MS, et al. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with Small-Vessel vasculitis. Am J Kidney Dis. 2003;42:1149-53.doi: 10.1053/j.ajkd.2003.08.015Levy JB, Hammad T, Coulthart A, et al. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004;66:1535-40. doi: 10.1111/j.1523-1755.2004.00917.xLevy JB, Hammad T, Coulthart A, et al. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004;66:1535-40. doi: 10.1111/j.1523-1755.2004.00917.xMetzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford). 2007;46:1087-91. doi: 10.1093/rheumatology/kem029Metzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford). 2007;46:1087-91. doi: 10.1093/rheumatology/kem029Stegeman CA, Tervaert JWC, de Jong PE, et al. Trimethoprimsulfamethoxazole (Co- trimoxazole) for the prevention of relapses of Wegener's granulomatosis. N Engl J Med. 1996;335:16-20. doi: 10.1056/NEJM199607043350103Stegeman CA, Tervaert JWC, de Jong PE, et al. Trimethoprimsulfamethoxazole (Co- trimoxazole) for the prevention of relapses of Wegener's granulomatosis. N Engl J Med. 1996;335:16-20. doi: 10.1056/NEJM199607043350103Springer J, Nutter B, Langford CA, et al. Granulomatosis with polyangiitis (Wegener's): impact of maintenance therapy duration. Medicine (Baltimore). 2014;93:82-90. doi: 10.1097/MD.0000000000000020Springer J, Nutter B, Langford CA, et al. Granulomatosis with polyangiitis (Wegener's): impact of maintenance therapy duration. Medicine (Baltimore). 2014;93:82-90. doi: 10.1097/MD.0000000000000020Walsh M, Merkel PA, Mahr A, et al. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis. Arthritis Care Res (Hoboken). 2010;62:1166-73. doi: 10.1002/acr.20176Walsh M, Merkel PA, Mahr A, et al. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis. Arthritis Care Res (Hoboken). 2010;62:1166-73. doi: 10.1002/acr.20176Pagnoux C, Hogan SL, Chin H, et al. Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibodyassociated small-vessel vasculitis: comparison of two independent cohorts. Arthritis Rheum. 2008;58:2908-18. doi: 10.1002/art.23800Pagnoux C, Hogan SL, Chin H, et al. Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibodyassociated small-vessel vasculitis: comparison of two independent cohorts. Arthritis Rheum. 2008;58:2908-18. doi: 10.1002/art.23800Hellmich B, Flossmann O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibodyassociated vasculitis. Ann Rheum Dis. 2007;66:605-17. doi: 10.1136/ard.2006.062711Hellmich B, Flossmann O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibodyassociated vasculitis. Ann Rheum Dis. 2007;66:605-17. doi: 10.1136/ard.2006.062711Holle JU, Voigt C, Both M, et al. Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage. Rheumatology (Oxford). 2013;52:875-82. doi: 10.1093/rheumatology/kes382Holle JU, Voigt C, Both M, et al. Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage. Rheumatology (Oxford). 2013;52:875-82. doi: 10.1093/rheumatology/kes382Pullerits R, Ljevak M, Vikgren J, et al. Off-trial evaluation of the B cell-targeting treatment in the refractory cases of antineutrophil cytoplasmic antibodies (ANCA)- associated vasculitis: long-term follow-up from a single centre. Scand J Immunol. 2012;76:411-20. doi: 10.1111/j.1365-3083.2012.02747.xPullerits R, Ljevak M, Vikgren J, et al. Off-trial evaluation of the B cell-targeting treatment in the refractory cases of antineutrophil cytoplasmic antibodies (ANCA)- associated vasculitis: long-term follow-up from a single centre. Scand J Immunol. 2012;76:411-20. doi: 10.1111/j.1365-3083.2012.02747.xSeror R, Pagnoux C, Ruivard M, et al. Treatment strategies and outcome of induction- refractory Wegener's granulomatosis or microscopic polyangiitis: analysis of 32 patients with first-line induction-refractory disease in the WEGENT trial. Ann Rheum Dis. 2010;69:2125-30. doi: 10.1136/ard.2010.131953Seror R, Pagnoux C, Ruivard M, et al. Treatment strategies and outcome of induction- refractory Wegener's granulomatosis or microscopic polyangiitis: analysis of 32 patients with first-line induction-refractory disease in the WEGENT trial. Ann Rheum Dis. 2010;69:2125-30. doi: 10.1136/ard.2010.131953Fortin PM, Tejani AM, Bassett K, et al. Intravenous immunoglobulin as adjuvant therapy for Wegener's granulomatosis. Cochrane Database Syst Rev. 2013;1:CD007057. doi: 10.1002/14651858.CD007057.pub3Fortin PM, Tejani AM, Bassett K, et al. Intravenous immunoglobulin as adjuvant therapy for Wegener's granulomatosis. Cochrane Database Syst Rev. 2013;1:CD007057. doi: 10.1002/14651858.CD007057.pub3Тomasson G, Grayson PC, Mahr AD, et al. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis – a meta-analysis. Rheumatology (Oxford). 2012;51:100-9. doi: 10.1093/rheumatology/ker280Тomasson G, Grayson PC, Mahr AD, et al. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis – a meta-analysis. Rheumatology (Oxford). 2012;51:100-9. doi: 10.1093/rheumatology/ker280Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med. 2007;120:643.e9-14. doi: 10.1016/j.amjmed.2006.08.016Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med. 2007;120:643.e9-14. doi: 10.1016/j.amjmed.2006.08.016Mukhtyar C, Lee R, Brown D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68:1827-32. doi: 10.1136/ard.2008.101279Mukhtyar C, Lee R, Brown D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68:1827-32. doi: 10.1136/ard.2008.101279Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40:371-80. doi: 10.1002/art.1780400222Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40:371-80. doi: 10.1002/art.1780400222Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine. 2011;90:19-27. doi: 10.1097/MD.0b013e318205a4c6Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine. 2011;90:19-27. doi: 10.1097/MD.0b013e318205a4c6De Groot K, Gross WL, Herlyn K, Reinhold-Keller E. Development and validation of a disease extent index for Wegener's granulomatosis. Clin Nephrol. 2001;55(1):31-8.De Groot K, Gross WL, Herlyn K, Reinhold-Keller E. Development and validation of a disease extent index for Wegener's granulomatosis. Clin Nephrol. 2001;55(1):31-8.Alberici F, Smith RM, Jones RB, et al. Long-term follow-up of patients who received repeat-dose rituximab as maintenance therapy for ANCA-associated vasculitis. Rheumatology (Oxford). 2015;54:1153-60. doi: 10.1093/rheumatology/keu452Alberici F, Smith RM, Jones RB, et al. Long-term follow-up of patients who received repeat-dose rituximab as maintenance therapy for ANCA-associated vasculitis. Rheumatology (Oxford). 2015;54:1153-60. doi: 10.1093/rheumatology/keu452Suppiah R, Judge A, Batra R, et al. A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis. Arthritis Care Res (Hoboken). 2011;63:588-96. doi: 10.1002/acr.20433Suppiah R, Judge A, Batra R, et al. A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis. Arthritis Care Res (Hoboken). 2011;63:588-96. doi: 10.1002/acr.20433

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