Pathogenesis and treatment progress of antineutrophil cytoplasmic antibody-associated vasculitis

doi:10.12092/j.issn.1009-2501.2018.02.022

Abstract

Abstract:

Vasculitis is an autoimmune disease which may cause nonspecific inflammation and necrosis of the blood vessels. According to degree of the vascular involvement, vasculitis divides into small vasculitis, moderate vasculitis and large vasculitis. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) which belongs to the small vasculitis includes granulomatous polyangiitis (GPA) microscopic polyangiitis (MPA) and eosinophilic granulomatous polyangiitis (EGPA). Different organs of the whole body could be involved and the active period is extremely dangerous. Untimely treatment can result in high mortality. This paper briefly reviews the pathogenesis and treatment progress of AAV.

Key words: vasculitis antineutrophil cytoplasmic antibody-associated (AAV), cyclophosphamide (CTX), rituximab (RTX)

CLC Number:

R593.27

YU Shuyuan, ZHENG Lin, HAN Fei. Pathogenesis and treatment progress of antineutrophil cytoplasmic antibody-associated vasculitis[J]. Chinese Journal of Clinical Pharmacology and Therapeutics, 2018, 23(2): 235-240.

References

［1］Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides［J］.Arthritis Rheum,2013,65(3):1-11.
［2］Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa［J］.Am J Med 1967,43(1):8-14.
［3］Mukhtyar C, Flossmann O, Hellmich B, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force［J］.Ann Rheum Dis 2008, 67(7):1004-1010.
［4］Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS)［J］.Clin Exp Nephrol,2013,17(5):619-621．
［5］Pagnoux C. Churg-Strauss syndrome: evolving concepts ［J］.Discov Med, 2010, 9(46):243-252.
［6］Wieczorek S, Holle JU, Epplen JT. Recent progress in the genetics of Wegener's granulomatosis and Churg-Strauss syndrome ［J］.Curr Opin Rheumatol,2010,22(1):8-14.
［7］Xie G, Roshandel D, Sherva R, et al. Association of granulomatosis with polyangiitis (Wegener's) with HLA-DPB1*04 and SEMA6A gene variants: evidence from genome-wide analysis［J］.Arthritis Rheum,2013,65(9):2457-2468.
［8］Rahmattulla C, Mooyaart AL, van Hooven D, et al. Genetic variants in ANCA-associated vasculitis: a meta-analysis［J］.Ann Rheum Dis,2016,75(9):1687-1692.
［9］Vaglio A, Casazza I, Grasselli C,et al.Churg-Strauss syndrome［J］.Kidney Int,2009,76(9):1006-1011.
［10］Jarrot PA, Kaplanski G. Pathogenesis of ANCA-associated vasculitis: An update ［J］.Autoimmun Rev,2016,15(7):704-713.
［11］Bansal PJ, Tobin MC. Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement［J］.Ann Allergy Asthma Immunoll,2004,93(4):398-401.
［12］Roth AJ, Ooi JD, Hess JJ, et al. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis［J］.J Clin Invest,2013,123(4):1773-783.
［13］Preston GA, Falk RJ. ANCA signaling: not just a matter of respiratory burst［J］.Kidney Int,2001,59(5):1981-1982.
［14］Kallenberg CG, Stegeman CA, Abdulahad WH, et al. Pathogenesis of ANCA-associated vasculitis: new possibilities for intervention ［J］.Am J Kidney Dis, 2013, 62(6):1176-1187.
［15］Gapud EJ, Seo P, Antiochos B. ANCA-associated vasculitis pathogenesis: A commentary ［J］.Curr Rheumatol Rep,2017,19(4):15.
［16］Kallenberg CG, Heeringa P. Complement is crucial in the pathogenesis of ANCA-associated vasculitis［J］.Kidney Int,2013,83(1):16-18.
［17］Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis［J］.Ann Rheum Dis,2016,75(9):1583-1594.
［18］McGregor JG, Hogan SL, Hu Y, et al. Glucocorticoids and relapse and infection rates in anti-neutrophil cytoplasmic antibody disease［J］.Clin J Am Soc Nephrol,2012,7(2):240-247.
［19］Stone JH, Merke PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis［J］.N Engl J Med,2010,363(3):221-232.
［20］Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis［J］.N Engl J Med,2010,363(3):211-220.
［21］van Daalen EE, Rizzo R, Kronbichler A, et al. Effect of rituximab on malignancy risk in patients with ANCA-associated vasculitis［J］.Ann Rheum Dis,2017,76(6):1064-1069.
［22］Unizony S, Villarrea M, Miloslavsky EM,et al. Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type［J］.Ann Rheum Dis,2016,75(6):1166-1169.
［23］Turner-Stokes T, Sandhu E, Pepper RJ, et al. Induction treatment of ANCA-associated vasculitis with a single dose of rituximab［J］.Rheumatology (Oxford),2014,53(8):1395-1403.
［24］Sznajd J, Mukhtyar C. How to treat ANCA-associated vasculitis: practical messages from 2016 EULAR/ERA-EDTA recommendations［J］.Pol Arch Med Wewn, 2016, 126(10):781-788.
［25］Singer O, McCune WJ. Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis［J］.Curr Opin Rheumatol,2017,29(3):248-253.
［26］Pagnoux C, Quéméneur T, Ninet J, et al. Treatment of systemic necrotizing vasculitides in patients aged sixty-five years or older: results of a multicenter, open-label, randomized controlled trial of corticosteroid and cyclophosphamide-based induction therapy ［J］.Arthritis Rheumatol,2015,67(4):1117-1127.
［27］Navarra SV, Guzmán RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial ［J］.Lancet 2011, 377(9767):721-731.
［28］Lenert A, Lenert P. Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents ［J］.Drug Des Devel Ther, 2015,9:333-347.
［29］Lenert A, Niewold TB, Lenert P. Spotlight on blisibimod and its potential in the treatment of systemic lupus erythematosus: evidence to date［J］.Drug Des Devel Ther,2017,11:747-757.
［30］Herrmann K, Gross WL, Moosig F. Extended follow-up after stopping mepolizumab in relapsing/refractory Churg-Strauss syndrome［J］.Clin Exp Rheumatol,2012,30(70):S62-S65.
［31］Walsh M, Chaudhry A, Jayne D. Long-term follow-up of relapsing/refractory anti-neutrophil cytoplasm antibody associated vasculitis treated with the lymphocyte depleting antibody alemtuzumab (CAMPATH-1H)［J］.Ann Rheum Dis,2008,67(9):1322-1327.
［32］Langford CA, Monach PA, Specks U, et al. An open-label trial of abatacept (CTLA4-IG) in non-severe relapsing granulomatosis with polyangiitis (Wegener's) ［J］.Ann Rheum Dis,2014,73(7):1376-1379.
［33］Schmitt WH, Hagen EC, Neumann I, et al．Treatment of refmctory wegener's granulomatosis with antithymocyte globulin(ATG)：an open study in 15 patients［J］.Kidney Int,2004,65(4):1440-1448.
［34］Birck R, Warnatz K, Lorenz HM, et al. 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis：a six-month open-label trial to evaluate safety and efficacy［J］.J Am Soc Nephrol,2003,14(2):440-447．
［35］Flossmann O, Jayne DR. Long-term treatment of relapsing Wegener's granulomatosis with 15-deoxyspergualin ［J］.Rheumatology (Oxford), 2010, 49(3):556-562.
［36］Sakai R, Kondo T, Kikuchi J, et al. Corticosteroid-free treatment of tocilizumab monotherapy for microscopic polyangiitis: a single-arm, single-center, clinical trial ［J］.Mod Rheumatol, 2016,26(6):900-907.
［37］Berti A, Cavalli G, Campochiaro C, et al. Interleukin-6 in ANCA-associated vasculitis: Rationale for successful treatment with tocilizumab ［J］.Semin Arthritis Rheum,2015,45(1):48-54.
［38］Sakai R, Kondo T, Kurasawa T, et al. Current clinical evidence of tocilizumab for the treatment of ANCA-associated vasculitis: a prospective case series for microscopic polyangiitis in a combination with corticosteroids and literature review ［J］.Clin Rheumatol,2017,36(10):2383-2392.
［39］Jayne DR, Bruchfeld AN, Harper L, et al. Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis［J］.J Am Soc Nephrol,2017,28(9):2756-2767.
［40］Hiemstra TF, Walsh M, Mahr A,et al.Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial［J］.JAMA,2010,304(21):2381-2388.
［41］Jones R, Harper L, Ballarin J, et al. A randomized trial of mycophenolate mofetil versus cyclophosphamide for remission induction of ANCA-associated vasculitis: "MYCYC". On behalf of the European vasculitis study group［J］.La Presse Med,2013,42(4):678-679.
［42］Chen Y, Gao E, Yang L, et al. Long-term outcome of mycophenolate mofetil treatment for patients with microscopic polyangiitis: an observational study in Chinese patients［J］.Rheumatol Int,2016,36(7):967-974.
［43］Cambridge G, Leandro MJ, Teodorescu M, et al. B cell depletion therapy in systemic lupus erythematosus: effect on autoantibody and antimicrobial antibody profiles［J］.Arthritis Rheum,2006,54(11):3612-3622.
［44］Thiel J, Rizzi M, Engesser M,et al. B cell repopulation kinetics after rituximabtreatment in ANCA-associated vasculitides compared to rheumatoid arthritis, and connective tissue diseases: a longitudinal observational study on 120 patients［J］.Arthritis Res Ther,2017,19(1):101.
［45］Robson J, Doll H, Suppiah R, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials［J］.Ann Rheum Dis,2015,74(1):177-184.
［46］Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis［J］.Ann Rheum Dis 2011,70(3):488-494.
［47］Suppiah R, Judge A, Batra R, et al. A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis［J］.Arthritis Care Res (Hoboken),2011,63(4):588-596.