Microscopic polyangiitis: Modern concepts and treatment options
I. T. Murkamilov,
K. A. Aitbaev,
V. V. Fomin,
I. O. Kudaibergenova,
F. A. Yusupov,
Zh. A. Murkamilova,
T. V. Beketova https://doi.org/10.47360/1995-4484-2021-608-614
Abstract
The article summarizes the modern concepts of microscopic polyangiitis (MPA), a primary ANCA-associated systemic necrotizing vasculitis without immune globulin deposition (pauci-immune) that affects mainly small vessels, while granulomatous inflammation is absent. Necrotizing glomerulonephritis is very common and pulmonary capillaritis often occurs. MPA can cause rapidly progressive damage to organ systems. The modern possibilities of MPA treatment, primarily anti-B cell therapy with rituximab, are discussed.
About the Authors
I. T. Murkamilov
I.K. Akhunbaev Kyrgyz State Medical Academy
Kyrgyzstan
Kyrgyzstan
720020, Kyrgyzstan, Bishkek, Akhunbaev str., 92
K. A. Aitbaev
Scientific and Research Institute of Molecular Biology and Medicine
Kyrgyzstan
Kyrgyzstan
720040, Kyrgyzstan, Bishkek, Togolok Moldo str., 3
V. V. Fomin
I.M. Sechenov First Moscow State Medical University of the Ministry of Health Care of Russian Federation (Sechenov University)
Russian Federation
Russian Federation
119991, Russian Federation, Moscow, Trubetskaya str., 8, building 2
I. O. Kudaibergenova
I.K. Akhunbaev Kyrgyz State Medical Academy
Kyrgyzstan
Kyrgyzstan
720020, Kyrgyzstan, Bishkek, Akhunbaev str., 92
F. A. Yusupov
Osh State University
Kyrgyzstan
Kyrgyzstan
723500, Kyrgyzstan, Osh, Lenin str. 331
Zh. A. Murkamilova
B.N. Yeltsin Kyrgyz-Russian Slavic University
Kyrgyzstan
Kyrgyzstan
720000, Kyrgyzstan, Bishkek, Kiev str. 44
T. V. Beketova
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Russian Federation
115522, Russian Federation, Moscow, Kashirskoye Highway, 34A
References
1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
2. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-1594. doi: 10.1136/annrheumdis-2016-209133
3. Beketova TV. Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies: Clinical features. Terapevticheskii arkhiv. 2015;87(5):33-46 (In Russ.). doi: 10.17116/terarkh201587533-46
4. Nguyen Y, Pagnoux C, Karras A, Quéméneur T, Maurier F, Hamidou M, et al. Microscopic polyangiitis: Clinical characteristics and long-term outcomes of 378 patients from the French Vasculitis Study Group Registry. J. Autoimmun. 2020;112:102467. doi: 10.1016/j.jaut.2020.102467
5. Wohlwill F. Über die nur mikroskopisch erkennbare Form der Periarteritis nodosa. Virchows Arch Pathol Anat Physiol. 1923;246:36.
6. Wainwright J, Davson J. The renal appearances in the microscopic form of periarteritis nodosa. J Pathol Bacteriol. 1950;62(2):189-196. doi: 10.1002/path.1700620206
7. Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533-553.
8. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: Presentation, pathology and prognosis. Q J Med. 1985;56(220):467-483.
9. van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet. 1985;1(8426):425-429. doi: 10.1016/s0140-6736(85)91147-x
10. Gross WL, Schmitt WH, Csernok E. ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol. 1993;91(1):1-12. doi: 10.1111/j.1365-2249.1993.tb03345.x
11. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an International Consensus Conference. Arthritis Rheum. 1994;37(2):187-192. doi: 10.1002/art.1780370206
12. Semenkova EN, Beketova TV, Kogan EA, Kozlovskaya LV, Mukhin NA. Modern concepts of microscopic polyarteritis. Terapevticheskii arkhiv. 1995;67(5):39-41 (In Russ.).
13. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66(2):222-227. doi: 10.1136/ard.2006.054593
14. Chung SA, Seo P. Microscopic polyangiitis. Rheum Dis Clin North Am. 2010;36(3):545-558. doi: 10.1016/j.rdc.2010.04.003
15. Fujimoto S, Uezono S, Hisanaga S, Fukudome K, Kobayashi S, Suzuki K, et al. Incidence of ANCA-associated primary renal vasculitis in the Miyazaki Prefecture: The first population based, retrospective, epidemiologic survey in Japan. Clin J Am Soc Nephrol. 2006;1(5):1016-1022. doi: 10.2215/CJN.01461005
16. Ormerod AS, Cook MC. Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South Wales. Intern Med J. 2008;38(11):816-823. doi: 10.1111/j.1445-5994.2008.01672.x
17. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-494. doi: 10.1136/ard.2010.137778
18. Shi J, Shen Q, Chen XM, Du XG. Clinical characteristics and outcomes in microscopic polyangiitis patients with renal involvement: a study of 124 Chinese patients. BMC Nephrol. 2019;20(1):339. doi: 10.1186/s12882-019-1535-3
19. Sugiyama K, Sada KE, Kurosawa M, Wada J, Makino H. Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan. Clin Exp Nephrol. 2013;17(1):51-58. doi: 10.1007/s10157-012-0651-1
20. Lane SE, Watts R, Scott DG. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. 2005;7(4):270-275. doi: 10.1007/s11926-005-0036-5
21. Comarmond C, Crestani B, Tazi A, Hervier B, Adam- Marchand S, Nunes H, et al. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: A series of 49 patients and review of the literature. Medicine (Baltimore). 2014;93(24):340-349. doi: 10.1097/MD.0000000000000217
22. Beketova TV. Diagnostic algorithm for antineutrophil cytoplasmic antibody-associated systemic vasculitis. Terapevticheskii arkhiv. 2018;90(5):13-21 (In Russ.). doi: 10.26442/terarkh201890513-21
23. Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, et al. Genetically distinct subsets within ANCA-associated vascuonline litis. N Engl J Med. 2012;367(3):214-223. doi: 10.1056/NEJMoa1108735
24. Tsuchiya N, Kobayashi S, Hashimoto H, Ozaki S, Tokunaga K. Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese. Genes Immun. 2006;7(1):81-84. doi: 10.1038/sj.gene.6364262
25. Rahmattulla C, Mooyaart AL, van Hooven D, Schoones JW, Bruijn JA, Dekkers OM, et al. Genetic variants in ANCAassociated vasculitis: A meta-analysis. Ann Rheum Dis. 2016;75(9):1687-1692. doi: 10.1136/annrheumdis-2015-207601
26. Pervakova MYu, Chudinov AL, Lapin SV, Belyaeva IB, Mazurov VI, Blinova TV, et al. The diagnostic and clinical value of determination of α1-antitrypsin phenotype in systemic vasculitides. Nauchcno-Prakticheskaya Revmatologia = Rheumatology Science and Practice. 2017;55(2):164-168 (In Russ.). doi: 10.14412/1995-4484-2017-164-168
27. Hagen EC, Ballieux BE, van Es LA, Daha MR, van der Woude FJ. Antineutrophil cytoplasmic autoantibodies: A review of the antigens involved, the assays, and the clinical and possible pathogenetic consequences. Blood. 1993;81(8):1996-2002.
28. Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Antineutrophil cytoplasmic antibodies: Current diagnostic and pathophysiological potential. Kidney Int. 1994;46(1):1-15. doi: 10.1038/ki.1994.239
29. Krumbholz M, Specks U, Wick M, Kalled SL, Jenne D, Meinl E. BAFF is elevated in serum of patients with Wegener’s granulomatosis. J Autoimmun. 2005;25(4):298-302. doi: 10.1016/j.jaut.2005.08.004
30. Witko-Sarsat V, Daniel S, Noël LH, Mouthon L. Neutrophils and B lymphocytes in ANCA-associated vasculitis. APMIS Suppl. 2009;127:27-31. doi: 10.1111/j.1600-0463.2009.02473.x
31. Taekema-Roelvink MEJ, Kooten CV, Kooij SV, Heemskerk E, Daha MR. Proteinase 3 enhances endothelial monocyte chemoattractant protein-1 production and induces increased adhesion of neutrophils to endothelial cells by upregulating intercellular cell adhesion molecule-1. J Am Soc Nephrol. 2001;12(5):932-940. doi: 10.1681/ASN.V125932
32. Mayet WJ, Schwarting A, Meyer zum Büschenfelde KH. Cytotoxic effects of antibodies to proteinase 3 (C-ANCA) on human endothelial cells. Clin Exp Immunol. 1994;97(3):458-465. doi: 10.1111/j.1365-2249.1994.tb06110.x
33. Foucher P, Heeringa P, Petersen AH, Huitema MG, Brouwer E, Tervaert JW, et al. Antimyeloperoxidase-associated lung disease. An experimental model. Am J Respir Crit Care Med. 1999;160(3):987-994. doi: 10.1164/ajrccm.160.3.9807139
34. Xiao H, Heeringa P, Liu Z, Huugen D, Hu P, Maeda N, et al. The role of neutrophils in the induction of glomerulonephritis by anti-myeloperoxidase antibodies. Am J Pathol. 2005;167(1):39-45. doi: 10.1016/S0002-9440(10)62951-3
35. Schreiber A, Xiao H, Jennette JC, Schneider W, Luft FC, Kettritz R. C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis. J Am Soc Nephrol. 2009;20(2):289-298. doi: 10.1681/ASN.2008050497
36. Hao J, Meng LQ, Xu PC, Chen M, Zhao MH. p38MAPK, ERK and PI3K signaling pathways are involved in C5a-primed neutrophils for ANCA-mediated activation. PLoS ONE. 2012;7(5):e38317. doi: 10.1371/journal.pone.0038317
37. Antovic A, Mobarrez F, Manojlovic M, Soutari N, De Porta Baggemar V, Nordin A, et al. Microparticles expressing myeloperoxidase and complement C3a and C5a as markers of renal involvement in antineutrophil cytoplasmic antibody-associated vasculitis. J Rheumatol. 2020;47(5):714-721. doi: 10.3899/jrheum.181347
38. Jayne DRW, Bruchfeld AN, Harper L, Schaier M, Venning MC, Hamilton P, et al. Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis. J Am Soc Nephrol. 2017;28(9):2756-2767. doi: 10.1681/ASN.2016111179
39. Hu P, Su H, Xiao H, Gou SJ, Herrera CA, Alba MA, et al. Kinin B1 receptor is important in the pathogenesis of myeloperoxidase-specific ANCA GN. J Am Soc Nephrol.2020;31:297-307. doi: 10.1681/ASN.2019010032
40. Yamagata M, Ikeda K, Tsushima K, Iesato K, Abe M, Ito T, et al. Prevalence and responsiveness to treatment of lung abnormalities on chest computed tomography in patients with microscopic polyangiitis: A multicenter, longitudinal, retrospective study of one hundred fifty consecutive hospital-based Japanese patients. Arthritis Rheumatol. 2016;68(3):713-723. doi: 10.1002/art.39475
41. Shchegoleva EM, Bulanov NM, Vinogradova ES, Zykova AS, Novikov PI, Moiseev SV. Clinical variants and outcomes of microscopic polyangiitis. Clinical Pharmacology and Therapy. 2018;27(3):35-40 (In Russ.).
42. Hirayama K, Kobayashi M, Usui J, Arimura Y, Sugiyama H, Nitta K, et al. Pulmonary involvements of antineutrophil cytoplasmic autoantibody-associated renal vasculitis in Japan. Nephrol Dial Transplant. 2015;30(Suppl 1):i83-i93. doi: 10.1093/ndt/gfu385
43. Nada AK, Torres VE, Ryu JH, Lie JT, Holley KE. Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. Mayo Clin Proc. 1990;65(6):847-856. doi: 10.1016/s0025-6196(12)62575-0
44. Kida T, Tanaka T, Yokota I, Tamagaki K, Sagawa T, Kadoya M, et al. Association between preexisting lung involvements and the risk of diffuse alveolar hemorrhage in patients with microscopic polyangiitis: A multi-center retrospective cohort study. Mod Rheumatol. 2020;30(2):338-344. doi: 10.1080/14397595.2019.1601855
45. Frolova NF, Volgina GV, Biryukova LS, Stoplyarevich ES, Tomilina NA. Clinical and morphological correlations and prognosis in patients with different histomorphological variants of rapidly progressive ANCA-associated glomerulonephritis. Lechebnoe delo. 2016;4:68-77 (In Russ.).
46. Ahn JK, Hwang JW, Lee J, Jeon CH, Cha HS, Koh EM. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int. 2012;32(10):2979-2986. doi: 10.1007/s00296-011-2079-4
47. Arienti F, Franco G, Monfrini E, Santaniello A, Bresolin N, Saetti MC, et al. Microscopic polyangiitis with selective involvement of Central and Peripheral Nervous System: A case report. Front Neurol. 2020;11:269. doi: 10.3389/fneur.2020.00269
48. Tauseef A, Asghar MS, Amir M, Zafar M, Anum A, Alvi H, et al. Microscopic polyangiitis: An incidental finding in a patient with stroke. J Community Hosp Intern Med Perspect. 2020;10(1):50-54. doi: 10.1080/20009666.2020.1718479
49. Shchegoleva EM, Zykova AS, Bulanov NM, Novikov PI, Moiseev SV, Mukhin NA. Modern approaches to the diagnosis and treatment of microscopic polyangiitis. Clinical Medicine (Russian Journal). 2018;96(1):66-72 (In Russ.)]. doi: 10.18821/0023-2149-2018-96-1-66-72
50. Frolova NF, Korsakova LV, Stolyarevich ES, Nikonorova NO, Beketova TV. The onset of ANCA-associated Научно-практическая ревматология. 2021;59(5):608–614 systemic vasculitis masking rheumatoid arthritis. Nauchcno-Prakticheskaya Revmatologia = Rheumatology Science and Practice. 2015;53(6):653-656 (In Russ.). doi: 10.14412/1995-4484-2015-653-656
51. Pagnoux C, Seror R, Bérezné A, Rouabhia S, Goulvestre C, Guillevin L. Remittent non-destructive polysynovitis in P-ANCApositive vasculitis patients with anti-CCP antibodies. Joint Bone Spine. 2010;77(6):604-607. doi: 10.1016/j.jbspin.2010.02.013
52. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, et al. Microscopic polyangiitis: Clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42(3):421-430. doi: 10.1002/1529-0131(199904)42:3<421::AIDANR5> 3.0.CO;2-6
53. Rodgers H, Guthrie JA, Brownjohn AM, Turney JH. Microscopic polyarteritis: Clinical features and treatment. Postgrad Med J. 1989;65(766):515-518. doi: 10.1136/pgmj.65.766.515
54. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: Clinical features and mortality. QJM. 2005;98(2):97-111. doi: 10.1093/qjmed/hci015
55. Zakharova EV, Yakovlev VN, Vinogradova OV, Sheikh ZhV, Zhidkova NV, Alekseev VG. ANCA-associated vasculitis affecting lungs and kidneys: Clinical and morphological characteristic, treatment, outcomes. Clinical Medicine (Russian Journal), 2013;91(7):38-42 (In Russ.).
56. Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med.2020;382(7):622-631. doi: 10.1056/NEJMoa1803537
57. Beketova TV, Nasonov EL. Ten-year experience with rituximab for induction and maintenance therapy in patients with ANCA-associated systemic vasculitis. Modern Rheumatology Journal. 2020;14(1):12-19 (In Russ.). doi: 10.14412/1996-7012-2020-1-12-19
58. Moiseev S, Novikov P, Bulanov N, Zykova A, Safonova E. Assessing cardiovascular risk in patients with antineutrophil cytoplasmic antibody-associated vasculitis: Comment on the article by Wallace et al. Arthritis Rheumatol. 2020;72(1):200-201. doi: 10.1002/art.41080
59. Murkamilov I, Aitbaev K, Kudaibergenova I, Fomin V, Yusupov F. Microscopic polyangiitis: diagnostic and therapeutic approaches to treatment (literature review and clinical case description. The Scientific Heritage. 2020;48-2:26-34 (In Russ.).
60. Beketova TV. New treatments for systemic vasculitis: Focus on avacopan, an oral selective C5a receptor inhibitor. Clinical Pharmacology and Therapy. 2019;28(1):75-79 (In Russ.). doi: 10.32756/0869- 5490-2019-1-75-79
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Murkamilov I.T., Aitbaev K.A., Fomin V.V., Kudaibergenova I.O., Yusupov F.A., Murkamilova Zh.A., Beketova T.V. Microscopic polyangiitis: Modern concepts and treatment options. Rheumatology Science and Practice. 2021;59(5):608-614. (In Russ.) https://doi.org/10.47360/1995-4484-2021-608-614
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