Developments in researches on pathogenesis of immune-mediated necrotizing myopathy_West China Medical Journal

Authors：

LIU Tao , LIU Huan , LIN Sang , HUANG Yupeng ,  XIE Qibing

Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

XIE Qibing, Email: xieqibing1971@163.com

Keywords：

Immune-mediated necrotizing myopathy; Autoantibody; Pathogenesis; Autoimmunity

DOI：

10.7507/1002-0179.201811173

Video：

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Abstract Full text Figures/Tables Video References Cited by

Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness with high serum muscle enzyme levels, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. The mechanism of necrotizing myopathy remains unclear. The new European Neuromuscular Centre criteria divides IMNM into three distinct subtypes according to different autoantibodies, which reminds us antibodies may be involved in the pathogenesis of IMNM and different subtypes may have different pathogenesis. This review summarizes the current understanding of the pathogenesis of IMNM.

Citation： LIU Tao, LIU Huan, LIN Sang, HUANG Yupeng, XIE Qibing. Developments in researches on pathogenesis of immune-mediated necrotizing myopathy. West China Medical Journal, 2018, 33(12): 1549-1553. doi: 10.7507/1002-0179.201811173 Copy

1.	McCombs RP, MacMahon HE. Dermatomyositis associates with metastasizing bronchogenic carcinoma; a clinicopathological conference. Med Clin North Am, 1947, 31(5): 1148-1162.
2.	Krolikowska W, Pawlikowski M, Prusinski A, et al. A case of non specific myopathy with characteristics of necrotizing myopathy. Neurol Neurochir Psychiatr Pol, 1963, 13: 365-367.
3.	Emslie-Smith AM, Engel AG. Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration. Neurology, 1991, 41(6): 936-939.
4.	Allenbach Y, Mammen AL, Benveniste O, et al. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14-16 October 2016. Neuromuscul Disord, 2018, 28(1): 87-99.
5.	Kishi T, Rider LG, Pak K, et al. Association of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies with DRB1*07:01 and severe myositis in juvenile myositis patients. Arthritis Care Res (Hoboken), 2017, 69(7): 1088-1094.
6.	SEARCH Collaborative Group, Link E, Parish S, et al. SLCO1B1 variants and stain induced myopathy: a genome wide study. N Engl J Med, 2008, 359(8): 789-799.
7.	Patel J, Superko HR, Martin SS, et al. Genetic and immunologic susceptibility to statin-related myopathy. Atherosclerosis, 2015, 240(1): 260-271.
8.	Thompson PD, Panza G, Zaleski A, et al. Statin-associated side effects. JACC, 2016, 67(20): 2359-2410.
9.	Muntean DM, Thompson PD, Catapano AL, et al. Statin-associated myopathy and the quest for biomarkers: can we effectively predict statin-associated muscle symptoms?. Drug Discov Today, 2017, 22(1): 85-96.
10.	Leff RL, Burgess SH, Miller FW, et al. Distinct seasonal patterns in the onset of adult idiopathic inflammatory athy in patients with anti-Jo-1 and anti-signal recognition particle autoa-ntibodies. Arthritis Rheum, 1991, 34(11): 1391-1396.
11.	Allenbach Y, Keraen J, Bouvier AM, et al. High risk of cancer in autoimmune necrotizing myopathies: usefulness of myositis specific antibody. Brain, 2016, 139(8): 2131-2135.
12.	Christopher-Stine L, Casciola RL, Hong G, et al. A Noel autoantibody recognizing 200-kd and 100-kd proteins is associate-ed with an immune-mediated necrotizing myopathy. Arthritis Rheum, 2010, 62(9): 2757-2766.
13.	Mammen AL, Chung T, Christopher-Stine L, et al. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum, 2011, 63(3): 713-721.
14.	Istvan ES, Palnitkar M, Buchanan SK, et al. Crystal structure of the catalytic portion of human HMG-CoA reductase: insights into regulation of activity and catalysis. EMBO J, 2000, 19(5): 819-830.
15.	Brown MS, Faust JR, Goldstein JL, et al. Induction of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts incubated with compactin (ML-236B), a competitive inhibitor of the reductase. J Biol Chem, 1978, 253(4): 1121-1128.
16.	Nakanishi M, Goldstein JL, Brown MS, et al. Multivalent control of 3-hydroxy-3-methylglutaryl coenzyme A reductase. Mevalon-ate-derived product inhibits translation of mRNA and accelerates degradation of enzyme. J Biol Chem, 1988, 263(18): 8929-8937.
17.	Morikawa S, Murakami T, Yamazaki H, et al. Analysis of the global RNA expression profiles of skeletal muscle cells treated with statins. J Atheroscler Thromb, 2005, 12(3): 121-131.
18.	Joseph CG, Darrah E, Shah AA, et al. Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science, 2014, 343(6167): 152-157.
19.	Arouche-Delaperche L, Allenbach Y, Amelin D, et al. Pathogenic role of anti-signal recognition protein and anti-3-hydroxy-3-met-hylglutaryl CoA reductase antibodies in necrotizing myopathies: myofiber atrophy and impairment of muscle regeneration in necrotizeng autoimmune myopathies. Ann Neurol, 2017, 81(4): 538-548.
20.	Allenbach Y, Arouche-Delaperche L, Preusse C, et al. Necrosis in anti-SRP⁺ and anti-HMGCR⁺ myopathies: role of autoantibodies and complement. Neurology, 2018, 90(6): e507-e517.
21.	Reeves WH, Nigam SK, Blobel G. Human autoantibodies reactive with the signal-recognition particle. Proc Natl Acad Sci USA, 1986, 83(24): 9507-9511.
22.	Miller T, Al-Lozi MT, Lopate G, et al. Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiatry, 2002, 73(4): 420-428.
23.	汪茜, 蒲传强. 抗信号识别颗粒抗体阳性坏死性肌病研究进展. 国际神经病学神经外科学杂志, 2013, 40(4): 359-362.
24.	Römisch K, Miller FW, Dobberstein B, et al. Human autoantibodies against the 54 kDa protein of the signal recognition particle block function at multiple stages. Arthritis Res Ther, 2006, 8(2): R39.
25.	Racanelli V, Prete M, Musaraj G, et al. Autoantibodies to intracellular antigens: generation and pathogenetic role. Autoimmun Rev, 2011, 10(8): 503-508.
26.	Rojana-Udomsart A, Mitrpant C, Bundell C, et al. Complement-mediated muscle cell lysis: a possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM). J Neuroimmunol, 2013, 264(1/2): 65-70.
27.	Chung T, Christopher-Stine L, Paik JJ, et al. The composition of cellular infiltrates in anti-HMG-CoA reductase-associated myopathy. Muscle Nerve, 2015, 52(2): 189-195.
28.	Saclier M, Yacoub-Youssef H, Mackey AL, et al. Differentially activated macrophages orchestrate myogenic precursor cell fate during human skeletal muscle regeneration. Stem Cells, 2013, 31(2): 384-396.
29.	Knauβ S, Allenbach Y, Preuße C, et al. PD1 and PDL2axis confers T cell exhaustion in anti-SRP⁺ and anti-HMGCR⁺ myopathies. Neuromuscular Disorders, 2017(2017): S96-S249.
30.	Luo SL, Xie YG, Li Z, et al. Ecadherin expression and prognosis of oral cancer: a meta-analysis. Tumour Biol, 2014, 35(6): 5533-5537.
31.	许静, 蒲传强, 石强, 等. 高迁移率族蛋白 1 在免疫性坏死性肌病的发病机制作用//中华医学会第十七次全国神经病学学术会议论文汇编, 北京: 中华医学会, 2014: 132.
32.	Wan ZM, Zhang XJ, Peng AP, et al. TLR4-HMGB1 signaling pathway affects the inflammatory reaction of autoimmune myositis by regulating MHC-I. Int Immunopharmacol, 2016, 41(41): 74-81.
33.	张媛, 丁树哲. 运动性骨骼肌内质网应激与线粒体功能调控. 中国体育科技, 2017, 53(4): 91-96.
34.	Li CK, Knopp P, Moncrieffe H, et al. Overexpression of MHC class Ⅰ heavy chain protein in young skeletal muscle leads to severe myositis: implications for juvenile myositis. Am J Pathol, 2009, 175(3): 1030-1040.
35.	Needham M, Fabian V, Knezevic W, et al. Progressive myopathy with upregulation of MHC-Ⅰ associated with statin therapy. Neuromuscul Disord, 2006, 17(2): 194-200.
36.	Lemasters JJ. Selective mitochondrial autophagy, or mitophagy as a targeted defense against oxidative stress, mitochondrial dysfunction, and aging. Rejuvenation Res, 2005, 8(1): 3-5.
37.	Matsubara S, Bokuda K, Asano Y, et al. Mitophagy in three cases of immune-mediated necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies: ultrastructural and immune-histochemical studies. Neuromuscul Disord, 2018, 28(3): 283-288.
38.	Nogalska A, D’Agostino C, Terracciano C. Impaired autophagy in sporadic inclusion-body myositis and in endoplasmic reticulum stress-provoked cultured human muscle fibers. Am J Pathol, 2010, 177(3): 1377-1387.
39.	Egerer T, Martinez-Gamboa L, Dankof A, et al. Tissue-specific up-regulation of the proteasome subunit beta5i (LMP7) in Sjögren’s syndrome. Arthritis Rheum, 2006, 54(5): 1501-1508.
40.	Krause S, Kuckelkorn U, Dornri T, et al. Immuno-proteasome subunit L-MP2 expression is deregulated in Sjogren’s syndrome but not in other autoimmune disorders. Ann Rheum Dis, 2005, 65(8): 1021-1027.
41.	李珍, 罗玉凤, 曹金伶, 等. 免疫蛋白酶体亚基在干燥综合征唇腺中的表达. 中国医学科学院学报, 2011, 33(2): 146-150, 220.
42.	Bhattarai S, Ghannam K, Krause S, et al. The immunoproteasomes are key to regulate myokines and MHC class Ⅰ expression in idiopathic inflammatory myopathies. J Autoimmun, 2016, 75: 118-129.

1. McCombs RP, MacMahon HE. Dermatomyositis associates with metastasizing bronchogenic carcinoma; a clinicopathological conference. Med Clin North Am, 1947, 31(5): 1148-1162.
2. Krolikowska W, Pawlikowski M, Prusinski A, et al. A case of non specific myopathy with characteristics of necrotizing myopathy. Neurol Neurochir Psychiatr Pol, 1963, 13: 365-367.
3. Emslie-Smith AM, Engel AG. Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration. Neurology, 1991, 41(6): 936-939.
4. Allenbach Y, Mammen AL, Benveniste O, et al. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14-16 October 2016. Neuromuscul Disord, 2018, 28(1): 87-99.
5. Kishi T, Rider LG, Pak K, et al. Association of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies with DRB1*07:01 and severe myositis in juvenile myositis patients. Arthritis Care Res (Hoboken), 2017, 69(7): 1088-1094.
6. SEARCH Collaborative Group, Link E, Parish S, et al. SLCO1B1 variants and stain induced myopathy: a genome wide study. N Engl J Med, 2008, 359(8): 789-799.
7. Patel J, Superko HR, Martin SS, et al. Genetic and immunologic susceptibility to statin-related myopathy. Atherosclerosis, 2015, 240(1): 260-271.
8. Thompson PD, Panza G, Zaleski A, et al. Statin-associated side effects. JACC, 2016, 67(20): 2359-2410.
9. Muntean DM, Thompson PD, Catapano AL, et al. Statin-associated myopathy and the quest for biomarkers: can we effectively predict statin-associated muscle symptoms?. Drug Discov Today, 2017, 22(1): 85-96.
10. Leff RL, Burgess SH, Miller FW, et al. Distinct seasonal patterns in the onset of adult idiopathic inflammatory athy in patients with anti-Jo-1 and anti-signal recognition particle autoa-ntibodies. Arthritis Rheum, 1991, 34(11): 1391-1396.
11. Allenbach Y, Keraen J, Bouvier AM, et al. High risk of cancer in autoimmune necrotizing myopathies: usefulness of myositis specific antibody. Brain, 2016, 139(8): 2131-2135.
12. Christopher-Stine L, Casciola RL, Hong G, et al. A Noel autoantibody recognizing 200-kd and 100-kd proteins is associate-ed with an immune-mediated necrotizing myopathy. Arthritis Rheum, 2010, 62(9): 2757-2766.
13. Mammen AL, Chung T, Christopher-Stine L, et al. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum, 2011, 63(3): 713-721.
14. Istvan ES, Palnitkar M, Buchanan SK, et al. Crystal structure of the catalytic portion of human HMG-CoA reductase: insights into regulation of activity and catalysis. EMBO J, 2000, 19(5): 819-830.
15. Brown MS, Faust JR, Goldstein JL, et al. Induction of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts incubated with compactin (ML-236B), a competitive inhibitor of the reductase. J Biol Chem, 1978, 253(4): 1121-1128.
16. Nakanishi M, Goldstein JL, Brown MS, et al. Multivalent control of 3-hydroxy-3-methylglutaryl coenzyme A reductase. Mevalon-ate-derived product inhibits translation of mRNA and accelerates degradation of enzyme. J Biol Chem, 1988, 263(18): 8929-8937.
17. Morikawa S, Murakami T, Yamazaki H, et al. Analysis of the global RNA expression profiles of skeletal muscle cells treated with statins. J Atheroscler Thromb, 2005, 12(3): 121-131.
18. Joseph CG, Darrah E, Shah AA, et al. Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science, 2014, 343(6167): 152-157.
19. Arouche-Delaperche L, Allenbach Y, Amelin D, et al. Pathogenic role of anti-signal recognition protein and anti-3-hydroxy-3-met-hylglutaryl CoA reductase antibodies in necrotizing myopathies: myofiber atrophy and impairment of muscle regeneration in necrotizeng autoimmune myopathies. Ann Neurol, 2017, 81(4): 538-548.
20. Allenbach Y, Arouche-Delaperche L, Preusse C, et al. Necrosis in anti-SRP⁺ and anti-HMGCR⁺ myopathies: role of autoantibodies and complement. Neurology, 2018, 90(6): e507-e517.
21. Reeves WH, Nigam SK, Blobel G. Human autoantibodies reactive with the signal-recognition particle. Proc Natl Acad Sci USA, 1986, 83(24): 9507-9511.
22. Miller T, Al-Lozi MT, Lopate G, et al. Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiatry, 2002, 73(4): 420-428.
23. 汪茜, 蒲传强. 抗信号识别颗粒抗体阳性坏死性肌病研究进展. 国际神经病学神经外科学杂志, 2013, 40(4): 359-362.
24. Römisch K, Miller FW, Dobberstein B, et al. Human autoantibodies against the 54 kDa protein of the signal recognition particle block function at multiple stages. Arthritis Res Ther, 2006, 8(2): R39.
25. Racanelli V, Prete M, Musaraj G, et al. Autoantibodies to intracellular antigens: generation and pathogenetic role. Autoimmun Rev, 2011, 10(8): 503-508.
26. Rojana-Udomsart A, Mitrpant C, Bundell C, et al. Complement-mediated muscle cell lysis: a possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM). J Neuroimmunol, 2013, 264(1/2): 65-70.
27. Chung T, Christopher-Stine L, Paik JJ, et al. The composition of cellular infiltrates in anti-HMG-CoA reductase-associated myopathy. Muscle Nerve, 2015, 52(2): 189-195.
28. Saclier M, Yacoub-Youssef H, Mackey AL, et al. Differentially activated macrophages orchestrate myogenic precursor cell fate during human skeletal muscle regeneration. Stem Cells, 2013, 31(2): 384-396.
29. Knauβ S, Allenbach Y, Preuße C, et al. PD1 and PDL2axis confers T cell exhaustion in anti-SRP⁺ and anti-HMGCR⁺ myopathies. Neuromuscular Disorders, 2017(2017): S96-S249.
30. Luo SL, Xie YG, Li Z, et al. Ecadherin expression and prognosis of oral cancer: a meta-analysis. Tumour Biol, 2014, 35(6): 5533-5537.
31. 许静, 蒲传强, 石强, 等. 高迁移率族蛋白 1 在免疫性坏死性肌病的发病机制作用//中华医学会第十七次全国神经病学学术会议论文汇编, 北京: 中华医学会, 2014: 132.
32. Wan ZM, Zhang XJ, Peng AP, et al. TLR4-HMGB1 signaling pathway affects the inflammatory reaction of autoimmune myositis by regulating MHC-I. Int Immunopharmacol, 2016, 41(41): 74-81.
33. 张媛, 丁树哲. 运动性骨骼肌内质网应激与线粒体功能调控. 中国体育科技, 2017, 53(4): 91-96.
34. Li CK, Knopp P, Moncrieffe H, et al. Overexpression of MHC class Ⅰ heavy chain protein in young skeletal muscle leads to severe myositis: implications for juvenile myositis. Am J Pathol, 2009, 175(3): 1030-1040.
35. Needham M, Fabian V, Knezevic W, et al. Progressive myopathy with upregulation of MHC-Ⅰ associated with statin therapy. Neuromuscul Disord, 2006, 17(2): 194-200.
36. Lemasters JJ. Selective mitochondrial autophagy, or mitophagy as a targeted defense against oxidative stress, mitochondrial dysfunction, and aging. Rejuvenation Res, 2005, 8(1): 3-5.
37. Matsubara S, Bokuda K, Asano Y, et al. Mitophagy in three cases of immune-mediated necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies: ultrastructural and immune-histochemical studies. Neuromuscul Disord, 2018, 28(3): 283-288.
38. Nogalska A, D’Agostino C, Terracciano C. Impaired autophagy in sporadic inclusion-body myositis and in endoplasmic reticulum stress-provoked cultured human muscle fibers. Am J Pathol, 2010, 177(3): 1377-1387.
39. Egerer T, Martinez-Gamboa L, Dankof A, et al. Tissue-specific up-regulation of the proteasome subunit beta5i (LMP7) in Sjögren’s syndrome. Arthritis Rheum, 2006, 54(5): 1501-1508.
40. Krause S, Kuckelkorn U, Dornri T, et al. Immuno-proteasome subunit L-MP2 expression is deregulated in Sjogren’s syndrome but not in other autoimmune disorders. Ann Rheum Dis, 2005, 65(8): 1021-1027.
41. 李珍, 罗玉凤, 曹金伶, 等. 免疫蛋白酶体亚基在干燥综合征唇腺中的表达. 中国医学科学院学报, 2011, 33(2): 146-150, 220.
42. Bhattarai S, Ghannam K, Krause S, et al. The immunoproteasomes are key to regulate myokines and MHC class Ⅰ expression in idiopathic inflammatory myopathies. J Autoimmun, 2016, 75: 118-129.

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