Progress of research on pathogenesis of primary small vasculitis

doi:10.3877/cma.j.issn.2095-3216.2014.06.004

Abstract

Abstract:

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of common autoimmune diseases in which the kidney is one of the most involved organs with rapid progression and poor prognosis. ANCAs are specific serological markers of AAV. The pathogenesis of AAV has not yet been fully elucidated. However, the interaction among ANCA, neutrophil, and complement system plays a central role. Proinflammatory factors including C5a can prime neutrophils to express more target antigens for ANCA on neutrophil surface. ANCAs activate primed neutrophils to undergo a respiratory burst, degranulation, and produce neutrophil extracellular traps (NETs), which result in vascular endothelial cell injury. In addition,activation of neutrophil can activate complement alternative pathway to form C3 and C5 convertases and produce more C5a, thereby amplifying inflammation and leading to AAV. Besides, lymphocytes and anti-endothelial cell antibodies (AECA) also play an important role in pathogenesis of AAV.

Key words: Antineutrophil cytoplasmic antibody, Vasculitis, Neutrophil, Complement

Min Chen. Progress of research on pathogenesis of primary small vasculitis[J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2014, 03(06): 297-303.

/ / Recommend

Add to citation manager EndNote|Ris|BibTeX

URL: https://zhsbyjdzzz.cma-cmc.com.cn/EN/10.3877/cma.j.issn.2095-3216.2014.06.004

https://zhsbyjdzzz.cma-cmc.com.cn/EN/Y2014/V03/I06/297

References 68

12	Rarok AA, Stegeman CA, Limburg PC, et al. Neutrophil membrane expression of proteinase 3 (PR3) is related to relapse in PR3-ANCA-associated vasculitis [J]. J Am Soc Nephrol, 2002, 13(9):2232-2238.
13	Schreiber A, Otto B, Ju X, et al. Membrane proteinase 3 expression in patients with Wegener' s granulomatosis and in human hematopoietic stem cell-derived neutrophils [J]. J Am Soc Nephrol,2005, 16(7): 2216-2224.
14	Brachemi S, Mambole A, Fakhouri F, et al. Increased membrane expression of proteinase 3 during neutrophil adhesion in the presence of anti proteinase 3 antibodies [J]. J Am Soc Nephrol, 2007, 18(8): 2330-2339.
15	Bauer S, Abdgawad M, Gunnarsson L, et al. Proteinase 3 and CD177 are expressed on the plasma membrane of the same subset of neutrophils [J]. J Leukoc Biol, 2007, 81(2): 458-464.
16	von Vietinghoff S, Tunnemann G, Eulenberg C, et al. NB1 mediates surface expression of the ANCA antigen proteinase 3 on human neutrophils [J]. Blood, 2007, 109(10): 4487-4493.
17	Hu N, Westra J, Huitema MG, et al. Coexpression of CD177 and membrane proteinase 3 on neutrophils in antineutrophil cytoplasmic autoantibody-associated systemic vasculitis: anti-proteinase 3-mediated neutrophil activation is independent of the role of CD177-expressing neutrophils [J]. Arthritis Rheum, 2009, 60 (5):1548-1557.
18	Hess C, Sadallah S, Schifferli JA. Induction of neutrophil responsiveness to myeloperoxidase antibodies by their exposure to supernatant of degranulated autologous neutrophils [J]. Blood,2000, 96(8): 2822-2827.
19	Griffin SV, Chapman PT, Lianos EA, et al. The inhibition of myeloperoxidase by ceruloplasmin can be reversed by antimyeloperoxidase antibodies [J]. Kidney Int, 1999, 55 (3):917-925.
20	Zhang AH, Chen M, Gao Y, et al. Inhibition of oxidation activity of myeloperoxidase (MPO) by propylthiouracil (PTU) and anti-MPO antibodies from patients with PTU-induced vasculitis [J]. Clin Immunol, 2007, 122(2): 187-193.
21	van der Geld YM, Stegeman CA, Kallenberg CG. B cell epitope specificity in ANCA-associated vasculitis: does it matter [J] ? Clin Exp Immunol, 2004, 137(3): 451-459.
22	Brooks CJ, King WJ, Radford DJ, et al. IL-1 beta production by human polymorphonuclear leucocytes stimulated by anti-neutrophil cytoplasmic autoantibodies: relevance to systemic vasculitis [J].Clin Exp Immunol, 1996, 106(2): 273-279.
23	Cockwell P, Brooks CJ, Adu D, et al. Interleukin-8: A pathogenetic role in antineutrophil cytoplasmic autoantibodyassociated glomerulonephritis [J]. Kidney Int, 1999, 55(3):852-863.
24	Calderwood JW, Williams JM, Morgan MD, et al. ANCA induces beta2 integrin and CXC chemokine-dependent neutrophil-endothelial cell interactions that mimic those of highly cytokine-activated endothelium [J]. J Leukoc Biol, 2005, 77(1): 33-43.
25	De Bandt M, Meyer O, Hakim J, et al. Antibodies to proteinase-3 mediate expression of intercellular adhesion molecule-1 (ICAM-1,CD 54) [J]. Br J Rheumatol, 1997, 36(8): 839-846.
26	Yang JJ PG, Pendergraft WF, Segelmark M, et al. Internalization of proteinase 3 is concomitant with endothelial cell apoptosis and internalization of myeloperoxidase with generation of intracellular oxidants [J]. Am J Pathol, 2001, 158(2): 581-592.
27	Mayet W, Schwarting A, Barreiros AP, et al. Anti-PR-3 antibodies induce endothelial IL-8 release [J]. Eur J Clin Invest, 1999, 29(11): 973-979.
28	Berger SP, Seelen MA, Hiemstra PS, et al. Proteinase 3, the major autoantigen of Wegener's granulomatosis, enhances IL-8 production by endothelial cells in vitro [J]. J Am Soc Nephrol, 1996, 7(5):694-701.
29	Mayet WJ, Csernok E, Szymkowiak C, et al. Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis [J]. Blood, 1993, 82(4):1221-1229.
30	Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice [J]. J Clin Invest, 2002, 110 (7):955-963.
31	Huugen D, Xiao H, van Esch A, et al. Aggravation of antimyeloperoxidase antibody-induced glomerulonephritis by bacterial lipopolysaccharide: role of tumor necrosis factor-alpha [J]. Am J Pathol, 2005, 167(1): 47-58.
32	Little MA, Smyth CL, Yadav R, et al. Antineutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocytemicrovascular interactions in vivo [J]. Blood, 2005, 106(6):2050-2058.
33	Nolan SL, Kalia N, Nash GB, et al. Mechanisms of ANCAmediated leukocyte-endothelial cell interactions in vivo [J]. J Am Soc Nephrol, 2008, 19(5): 973-984.
34	Schreiber A, Xiao H, Falk RJ, et al. Bone marrow-derived cells are sufficient and necessary targets to mediate glomerulonephritis and vasculitis induced by anti-myeloperoxidase antibodies [J]. J Am Soc Nephrol, 2006, 17(12): 3355-3364.
35	Pfister H, Ollert M, Frohlich LF, et al. Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo [J]. Blood, 2004, 104(5):1411-1418.
36	Weidner S, Carl M, Riess R, et al. Histologic analysis of renal leukocyte infiltration in antineutrophil cytoplasmic antibodyassociated vasculitis: importance of monocyte and neutrophil infiltration in tissue damage [J]. Arthritis Rheum, 2004, 50(11):3651-3657.
37	Chen M, Wang F, Zhao MH. Circulating neutrophil gelatinaseassociated lipocalin: a useful biomarker for assessing disease activity of ANCA-associated vasculitis [J]. Rheumatology (Oxford), 2009,48(4): 355-358.
38	Xiao H, Heeringa P, Liu Z, et al. The role of neutrophils in the induction of glomerulonephritis by anti-myeloperoxidase antibodies[J]. Am J Pathol, 2005, 167(1): 39-45.
39	Kessenbrock K, Krumbholz M, Schonermarck U, et al. Netting neutrophils in autoimmune small-vessel vasculitis [J]. Nat Med,2009, 15(6): 623-625.
1	Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides[J]. Arthritis Rheum, 2013, 65(1): 1-11.
2	Schlieben DJ, Korbet SM, Kimura RE, et al. Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs [J].Am J Kidney Dis, 2005,45(4):758-761.
3	Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis [J]. J Am Soc Nephrol, 2007, 18(7): 2180-2188.
4	Mulder AH, Heeringa P, Brouwer E, et al. Activation of granulocytes by anti-neutrophil cytoplasmic antibodies (ANCA): a Fc gamma RII-dependent process [J]. Clin Exp Immunol, 1994,98(2): 270-278.
5	Falk RJ, Terrell RS, Charles LA, et al. Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro [J]. Proc Natl Acad Sci USA, 1990, 87(11):4115-4119.
6	Ewert BH, Jennette JC, Falk RJ. Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells [J]. Kidney Int, 1992, 41(2): 375-383.
7	Komocsi A, Lamprecht P, Csernok E, et al. Peripheral blood and granuloma CD4(+) CD28 (-) T cells are a major source of interferon-gamma and tumor necrosis factor-alpha in Wegener' s granulomatosis [J]. Am J Pathol, 2002, 160(5): 1717-1724.
8	Kocher M, Edberg JC, Fleit HB, et al. Antineutrophil cytoplasmic antibodies preferentially engage Fc gammaRIIIb on human neutrophils [J]. J Immunol, 1998, 161(12): 6909-6914.
9	Kettritz R, Jennette JC, Falk RJ. Crosslinking of ANCA-antigens stimulates superoxide release by human neutrophils [J]. J Am Soc Nephrol, 1997, 8(3): 386-394.
10	Schreiber A, Busjahn A, Luft FC, et al. Membrane expression of proteinase 3 is genetically determined [J]. J Am Soc Nephrol,2003, 14(1): 68-75.
11	Witko-Sarsat V, Lesavre P, Lopez S, et al. A large subset of neutrophils expressing membrane proteinase 3 is a risk factor for vasculitis and rheumatoid arthritis [J]. J Am Soc Nephrol, 1999,10(6): 1224-1233.
40	Chen M, Kallenberg CG. Novel territory for neutrophils in the pathogenesis of ANCA-associated vasculitides [J]. Nephrol Dial Transplant, 2009, 24(12): 3618-3620.
41	Xiao H, Schreiber A, Heeringa P, et al. Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies [J]. Am J Pathol, 2007, 170(1):52-64.
42	Gou S-J, Yuan J, Chen M, et al. Circulating complement activation in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis [J]. Kidney Int, 2013, 83(1): 129-137.
43	Gou SJ, Yuan J, Wang C, et al. Alternative complement pathway activation products in urine and kidneys of patients with ANCAassociated GN [J]. Clin J Am Soc Nephrol, 2013, 8(11):1884-1891.
44	Xing G-q, Chen M, Liu G, et al. Complement activation is involved in renal damage in human antineutrophil cytoplasmic autoantibody associated pauci-immune vasculitis [J]. J Clin Immun, 2009, 29(3): 282-291.
45	Hao J, Wang C, Yuan J, et al. A pro-inflammatory role of C5L2 in C5a-primed neutrophils for ANCA-induced activation [J]. PLoS One, 2013, 8(6): e66305.
46	Hao J, Chen M, Zhao MH. Involvement of protein kinase C in C5aprimed neutrophils for ANCA-mediated activation [ J]. Mol Immunol, 2013, 54(1): 68-73.
47	Camous L, Roumenina L, Bigot S, et al. Complement alternative pathway acts as a positive feedback amplification of neutrophil activation [J]. Blood, 2011, 117(4): 1340-1349.
48	Schreiber A, Xiao H, Jennette JC, et al. C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis [J]. J Am Soc Nephrol, 2009, 20(2): 289-298.
49	Xiao H, Dairaghi DJ, Powers JP, et al. C5a receptor (CD88)blockade protects against MPO-ANCA GN [J]. J Am Soc Nephrol,2014, 25(2): 225-231.
50	Yuan J, Gou S-J, Huang J, et al. C5a and its receptors in human anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis[J]. Arthritis Res Ther, 2012, 14(3): R140.
51	Hao J, Meng L-Q, Xu P-C, et al. p38MAPK, ERK and PI3K signaling pathways are involved in C5a-primed neutrophils for ANCA-mediated activation [J]. PLoS One, 2012, 7(5): e38317.
52	Guilpain P, Mouthon L. Antiendothelial cells autoantibodies in vasculitis-associated systemic diseases [ J]. Clin Rev Allergy Immunol, 2008, 35(1-2): 59-65.
53	Del Papa N, Guidali L, Sironi M, et al. Anti-endothelial cell IgG antibodies from patients with Wegener' s granulomatosis bind to human endothelial cells in vitro and induce adhesion molecule expression and cytokine secretion [J]. Arthritis Rheum, 1996, 39(5): 758-766.
54	Savage CO, Pottinger BE, Gaskin G, et al. Vascular damage in Wegener's granulomatosis and microscopic polyarteritis: presence of anti-endothelial cell antibodies and their relation to anti-neutrophil cytoplasm antibodies [J]. Clin Exp Immunol, 1991, 85(1):14-19.
55	Sebastian JK, Mahr AD, Ahmed SS, et al. Antiendothelial cell antibodies in patients with Wegener's granulomatosis: prevalence and correlation with disease activity and manifestations [ J]. J Rheumatol, 2007, 34(5): 1027-1031.
56	Yu F, Zhao MH, Zhang YK, et al. Anti-endothelial cell antibodies(AECA) in patients with propylthiouracil (PTU)-induced ANCA positive vasculitis are associated with disease activity [J]. Clin Exp Immunol, 2005, 139(3): 569-574.
57	Gobel U, Eichhorn J, Kettritz R, et al. Disease activity and autoantibodies to endothelial cells in patients with Wegener' s granulomatosis [J]. Am J Kidney Dis, 1996, 28(2): 186-194.
58	Holmen C, Christensson M, Pettersson E, et al. Wegener' s granulomatosis is associated with organ-specific antiendothelial cell antibodies [J]. Kidney Int, 2004, 66(3): 1049-1060.
59	Lamprecht P. Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides [J]. Clin Exp Immunol,2005, 141(2): 201-210.
60	Giscombe R, Nityanand S, Lewin N, et al. Expanded T cell populations in patients with Wegener' sgranulomatosis:characteristics and correlates with disease activity [J]. J Clin Immunol, 1998, 18(6): 404-413.
61	Clayton AR SC. Production of antineutrophil cytoplasm antibodies derived from circulating B cells in patients with systemic vasculitis[J]. Clin Exp Immunol, 2003, 132(1): 174-179.
62	Spiegelberg HL. Fc receptors for IgE and interleukin-4 induced IgE and IgG4 secretion [J]. J Invest Dermatol, 1990, 94(6 Suppl):49S-52S.
63	Vercelli D. One cytokine, two isotypes: a trojan horse, pandora's box, and an evolving paradigm [J]. Am J Respir Crit Care Med,2000, 162(3 Pt 2): S86-S90.
64	King WJ, Brooks CJ, Holder R, et al. T lymphocyte responses to anti-neutrophil cytoplasmic autoantibody (ANCA) antigens are present in patients with ANCA-associated systemic vasculitis and persist during disease remission [J]. Clin Exp Immunol, 1998, 112(3): 539-546.
65	Sanders JS, Stegeman CA, Kallenberg CG. The Th1 and Th2 paradigm in ANCA-associated vasculitis [J]. Kidney Blood Press Res, 2003, 26(4): 215-220.
66	Abdulahad WH, Stegeman CA, Limburg PC, et al. Skewed distribution of Th17 lymphocytes in patients with Wegener' s granulomatosis in remission [J]. Arthritis Rheum, 2008, 58(7):2196-2205.
67	Jin D, Zhang L, Zheng J, et al. The inflammatory Th 17 subset in immunity against self and non-self antigens [J]. Autoimmunity,2008, 41(2): 154-162.
68	Steinmetz OM, Velden J, Kneissler U, et al. Analysis and classification of B-cell infiltrates in lupus and ANCA-associated nephritis [J]. Kidney Int, 2008, 74(4): 448-457.

[1]	Xue Zhao, Meng Jin, Jianming Lai, Zanhua Rong, Xinli Bai. Childhood Behcet's disease complicated with intestinal pseudopolyps：two cases report and literature review [J]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2024, 20(04): 460-467.
[2]	Jinye Chen, Qianlong Ling, Bing Zhu, Jie Luo. Expression and clinical significance of complement B factor in colorectal cancer [J]. Chinese Archives of General Surgery(Electronic Edition), 2024, 18(03): 192-198.
[3]	Siping Hu, Xingyu Xiong, Hang Xu, Lu Yang. The mechanisms of senescence-associated secretory phenotype factors in the occurrence and progression of prostate cancer [J]. Chinese Journal of Endourology(Electronic Edition), 2024, 18(05): 425-434.
[4]	Liu Yang, Jia Chen, Yajuan Sun, Jiao Chen, Mingchao Tan, Mingfu Gong. Chest CT and clinical features of antineutrophil cytoplasmic antibody-associated vasculitis [J]. Chinese Journal of Lung Diseases(Electronic Edition), 2024, 17(05): 744-749.
[5]	Huiling He, Zubin Lu, Jiali Feng, Shengqiang Liang. Effect of preoperative peripheral blood NLR and PLR on postoperative colon cancer liver metastasis [J]. Chinese Journal of Hepatic Surgery(Electronic Edition), 2024, 13(05): 682-687.
[6]	Zeyun Zhao, Jiannan Li, Min Wang. Research progress of neutrophil extracellular traps in colorectal cancer [J]. Chinese Journal of Colorectal Diseases(Electronic Edition), 2024, 13(06): 524-528.
[7]	Xuan Tie, Xiaole Su, Lihua Wang. Progress of research on the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis [J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(06): 345-351.
[8]	Shaoqing Zhang, Yufeng Lv, Haixia Dong. The predictive effect of neutrophil percentage-to-albumin ratio for all-cause mortality in maintenance hemodialysis patients [J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(06): 321-326.
[9]	Yang Zhang, Shasha Luo, Wenjun Zou. New advances on the mechanism of age-related macular degeneration [J]. Chinese Journal of Ophthalmologic Medicine(Electronic Edition), 2024, 14(04): 240-246.
[10]	Wenkai Sun, Qing Shen, Li Hang, Yingchun Zhang. Relationship of FAR, NAR, CRP with the evaluation and prognosis of ulcerative colitis patients [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(05): 426-431.
[11]	Qiuzi Cui, Hongman Yao, Yingchun Ai. Analysis of the predictive value of monitoring NLR, PLR, CAR, albumin, serum calcium and blood glucose levels in acute kidney injury in patients with acute pancreatitis [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(03): 244-248.
[12]	Jing Gao, Tingting Xia. Evaluation value of serum lactate dehydrogenase level, neutrophil-lymphocyte ratio and plasma fibrinogen-prealbumin ratio on palliative chemotherapy effect and adverse reactions in patients with advanced colorectal cancer [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(03): 203-207.
[13]	Jie Zhang, Donghua Ji. Current status of the Indexes for predicting the severity of peripheral artery disease [J]. Chinese Journal of Interventional Radiology(Electronic Edition), 2024, 12(02): 176-179.
[14]	Jingni Xi, Na Li, Qi Zhang. Predictive value of neutrophil-to-lymphocyte ratio for progression of severe community-acquired pneumonia to sepsis in elderly patients [J]. Chinese Journal of Geriatrics Research(Electronic Edition), 2024, 11(03): 28-31.
[15]	Xin Tang, Wenhai Zhai, Runting Wang, Shengyu Zhou, Hang Jin. Research progress on the role of complement in ischemic stroke [J]. Chinese Journal of Cerebrovascular Diseases(Electronic Edition), 2024, 18(04): 382-392.

Please choose a citation manager

Content to export