Polymyositis and Dermatomyositis: Pathophysiology

Rheumatic Disease Clinics of North America

Volumn 37, Issue 2, 2011, Pages 159-171

  Nagaraju, Kanneboyina ^a   Lundberg, Ingrid E ^b  

^a GEORGE WASHINGTON UNIVERSITY MEDICAL CENTER   (United States)

^b KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

Author keywords

Adaptive; Autophagy and endoplasmic reticulum stress; Dermatomyositis; Innate and nonimmune pathways; Pathogenesis; Polymyositis; Skeletal muscle cell death

Indexed keywords

ALPHA INTERFERON; BETA INTERFERON; CYCLOOXYGENASE 1; CYCLOOXYGENASE 2; GLUCOSE 6 PHOSPHATE DEHYDROGENASE; HIGH MOBILITY GROUP B1 PROTEIN; HLA DR ANTIGEN; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN; INTERLEUKIN 15; INTERLEUKIN 18; INTERLEUKIN 1ALPHA; INTERLEUKIN 1BETA; JO 1 ANTIBODY; PROSTAGLANDIN E SYNTHASE 1; TUMOR NECROSIS FACTOR ALPHA;

ADAPTIVE IMMUNITY; ANTIGEN DETECTION; B LYMPHOCYTE; CD4+ T LYMPHOCYTE; CD8+ T LYMPHOCYTE; CELL DEATH; CELL INFILTRATION; CELL PROLIFERATION; CELL STRUCTURE; CELLULAR IMMUNITY; DERMATOMYOSITIS; ENDOPLASMIC RETICULUM STRESS; GENOTYPE; HUMAN; INNATE IMMUNITY; LYMPHOCYTE FUNCTION; LYMPHOCYTIC INFILTRATION; PATHOGENESIS; POLYMYOSITIS; PRIORITY JOURNAL; PROTEIN EXPRESSION; REVIEW; T LYMPHOCYTE; UPREGULATION;

ADAPTIVE IMMUNITY; AUTOANTIBODIES; AUTOIMMUNE DISEASES; AUTOIMMUNITY; DERMATOMYOSITIS; HUMANS; IMMUNITY, INNATE; MUSCLE WEAKNESS; MUSCLE, SKELETAL; NECROSIS; POLYMYOSITIS;

EID: 79953041968     PISSN: 0889857X     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.rdc.2011.01.002     Document Type: Review

References (83)

1. Dalakas M.C., Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003, 362:971-982.
2. Lundberg I.E. New possibilities to achieve increased understanding of disease mechanisms in idiopathic inflammatory myopathies. Curr Opin Rheumatol 2002, 14:639-642.
3. Lundberg I.E. The physiology of inflammatory myopathies: an overview. Acta Physiol Scand 2001, 171:207-213.
4. Nagaraju K. Immunological capabilities of skeletal muscle cells. Acta Physiol Scand 2001, 171:215-223.
5. Nagaraju K. Update on immunopathogenesis in inflammatory myopathies. Curr Opin Rheumatol 2001, 13:461-468.
6. Greenberg S.A., Pinkus J.L., Pinkus G.S., et al. Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol 2005, 57:664-678.
7. Lundberg I.E., Grundtman C. Developments in the scientific and clinical understanding of inflammatory myopathies. Arthritis Res Ther 2008, 10:220.
8. Nagaraju K., Casciola-Rosen L., Lundberg I., et al. Activation of the endoplasmic reticulum stress response in autoimmune myositis: potential role in muscle fiber damage and dysfunction. Arthritis Rheum 2005, 52:1824-1835.
9. Mierau R., Dick T., Bartz-Bazzanella P., et al. Strong association of dermatomyositis-specific Mi-2 autoantibodies with a tryptophan at position 9 of the HLA-DR beta chain. Arthritis Rheum 1996, 39:868-876.
10. Targoff I.N., Reichlin M. The association between Mi-2 antibodies and dermatomyositis. Arthritis Rheum 1985, 28:796-803.
11. Love L.A., Leff R.L., Fraser D.D., et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991, 70:360-374.
12. Gunawardena H., Betteridge Z.E., McHugh N.J. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford) 2009, 48:607-612.
13. Chinoy H., Fertig N., Oddis C.V., et al. The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis. Ann Rheum Dis 2007, 66:1345-1349.
14. Gunawardena H., Wedderburn L.R., North J., et al. Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology (Oxford) 2008, 47:324-328.
15. Targoff I.N., Mamyrova G., Trieu E.P., et al. A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum 2006, 54:3682-3689.
16. Greenberg S.A., Bradshaw E.M., Pinkus J.L., et al. Plasma cells in muscle in inclusion body myositis and polymyositis. Neurology 2005, 65:1782-1787.
17. Arahata K., Engel A.G. Monoclonal antibody analysis of mononuclear cells in myopathies. I: quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984, 16(2):193-208.
18. Bradshaw E.M., Orihuela A., McArdel S.L., et al. A local antigen-driven humoral response is present in the inflammatory myopathies. J Immunol 2007, 178:547-556.
19. Levine S.M., Raben N., Xie D., et al. Novel conformation of histidyl-transfer RNA synthetase in the lung: the target tissue in Jo-1 autoantibody-associated myositis. Arthritis Rheum 2007, 56:2729-2739.
20. Katsumata Y., Ridgway W.M., Oriss T., et al. Species-specific immune responses generated by histidyl-tRNA synthetase immunization are associated with muscle and lung inflammation. J Autoimmun 2007, 29:174-186.
21. Mammen A.L., Casciola-Rosen L.A., Hall J.C., et al. Expression of the dermatomyositis autoantigen Mi-2 in regenerating muscle. Arthritis Rheum 2009, 60:3784-3793.
22. Casciola-Rosen L., Nagaraju K., Plotz P., et al. Enhanced autoantigen expression in regenerating muscle cells in idiopathic inflammatory myopathy. J Exp Med 2005, 201:591-601.
23. Miller F.W., Waite K.A., Biswas T., et al. The role of an autoantigen, histidyl-tRNA synthetase, in the induction and maintenance of autoimmunity. Proc Natl Acad Sci U S A 1990, 87:9933-9937.
24. Stone K.B., Oddis C.V., Fertig N., et al. Anti-Jo-1 antibody levels correlate with disease activity in idiopathic inflammatory myopathy. Arthritis Rheum 2007, 56:3125-3131.
25. Miller F.W., Twitty S.A., Biswas T., et al. Origin and regulation of a disease-specific autoantibody response. Antigenic epitopes, spectrotype stability, and isotype restriction of anti-Jo-1 autoantibodies. J Clin Invest 1990, 85:468-475.
26. Eloranta M.L., Barbasso Helmers S., Ulfgren A.K., et al. A possible mechanism for endogenous activation of the type I interferon system in myositis patients with anti-Jo-1 or anti-Ro 52/anti-Ro 60 autoantibodies. Arthritis Rheum 2007, 56:3112-3124.
27. Barbasso Helmers S., Englund P., Engstrom M., et al. Sera from anti-Jo-1-positive patients with polymyositis and interstitial lung disease induce expression of intercellular adhesion molecule 1 in human lung endothelial cells. Arthritis Rheum 2009, 60:2524-2530.
28. Krystufkova O., Vallerskog T., Helmers S.B., et al. Increased serum levels of B cell activating factor (BAFF) in subsets of patients with idiopathic inflammatory myopathies. Ann Rheum Dis 2009, 68:836-843.
29. Page G., Chevrel G., Miossec P. Anatomic localization of immature and mature dendritic cell subsets in dermatomyositis and polymyositis: interaction with chemokines and Th1 cytokine-producing cells. Arthritis Rheum 2004, 50:199-208.
30. Greenberg S.A., Pinkus G.S., Amato A.A., et al. Myeloid dendritic cells in inclusion-body myositis and polymyositis. Muscle Nerve 2007, 35:17-23.
31. Emslie-Smith A.M., Engel A.G. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol 1990, 27:343-356.
32. Grundtman C., Tham E., Ulfgren A.K., et al. Vascular endothelial growth factor is highly expressed in muscle tissue of patients with polymyositis and patients with dermatomyositis. Arthritis Rheum 2008, 58:3224-3238.
33. Kissel J.T., Mendell J.R., Rammohan K.W. Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986, 314:329-334.
34. Goebels N., Michaelis D., Engelhardt M., et al. Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis. J Clin Invest 1996, 97:2905-2910.
35. Hohlfeld R., Engel A.G. Coculture with autologous myotubes of cytotoxic T cells isolated from muscle in inflammatory myopathies. Ann Neurol 1991, 29:498-507.
36. Fasth A.E., Dastmalchi M., Rahbar A., et al. T cell infiltrates in the muscles of patients with dermatomyositis and polymyositis are dominated by CD28null T cells. J Immunol 2009, 183:4792-4799.
37. Warrington K.J., Kent P.D., Frye R.L., et al. Rheumatoid arthritis is an independent risk factor for multi-vessel coronary artery disease: a case control study. Arthritis Res Ther 2005, 7:R984-R991.
38. Appay V., Dunbar P.R., Callan M., et al. Memory CD8+ T cells vary in differentiation phenotype in different persistent virus infections. Nat Med 2002, 8(4):379-385.
39. De Paepe B., Creus K.K., De Bleecker J.L. Role of cytokines and chemokines in idiopathic inflammatory myopathies. Curr Opin Rheumatol 2009, 21:610-616.
40. Tucci M., Quatraro C., Dammacco F., et al. Interleukin-18 overexpression as a hallmark of the activity of autoimmune inflammatory myopathies. Clin Exp Immunol 2006, 146:21-31.
41. Sugiura T., Harigai M., Kawaguchi Y., et al. Increased IL-15 production of muscle cells in polymyositis and dermatomyositis. Int Immunol 2002, 14:917-924.
42. Reid M.B., Lannergren J., Westerblad H. Respiratory and limb muscle weakness induced by tumor necrosis factor-alpha: involvement of muscle myofilaments. Am J Respir Crit Care Med 2002, 166:479-484.
43. Grundtman C., Bruton J., Yamada T., et al. Effects of HMGB1 on in vitro responses of isolated muscle fibers and functional aspects in skeletal muscles of idiopathic inflammatory myopathies. FASEB J 2010, 24:570-578.
44. Grundtman C., Salomonsson S., Dorph C., et al. Immunolocalization of interleukin-1 receptors in the sarcolemma and nuclei of skeletal muscle in patients with idiopathic inflammatory myopathies. Arthritis Rheum 2007, 56:674-687.
45. Furlan A., Botsios C., Ruffatti A., et al. Antisynthetase syndrome with refractory polyarthritis and fever successfully treated with the IL-1 receptor antagonist, anakinra: a case report. Joint Bone Spine 2008, 75:366-367.
46. Sugihara T., Sekine C., Nakae T., et al. A new murine model to define the critical pathologic and therapeutic mediators of polymyositis. Arthritis Rheum 2007, 56:1304-1314.
47. Korotkova M., Helmers S.B., Loell I., et al. Effects of immunosuppressive treatment on microsomal prostaglandin E synthase 1 and cyclooxygenases expression in muscle tissue of patients with polymyositis or dermatomyositis. Ann Rheum Dis 2008, 67:1596-1602.
48. Ronnblom L.E., Alm G.V., Oberg K.E. Possible induction of systemic lupus erythematosus by interferon-alpha treatment in a patient with a malignant carcinoid tumour. J Intern Med 1990, 227:207-210.
49. Kalkner K.M., Ronnblom L., Karlsson Parra A.K., et al. Antibodies against double-stranded DNA and development of polymyositis during treatment with interferon. QJM 1998, 91:393-399.
50. Dietrich L.L., Bridges A.J., Albertini M.R. Dermatomyositis after interferon alpha treatment. Med Oncol 2000, 17:64-69.
51. Tezak Z., Hoffman E.P., Lutz J.L., et al. Gene expression profiling in DQA1*0501+ children with untreated dermatomyositis: a novel model of pathogenesis. J Immunol 2002, 168:4154-4163.
52. Baechler E.C., Bauer J.W., Slattery C.A., et al. An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity. Mol Med 2007, 13:59-68.
53. Walsh R.J., Kong S.W., Yao Y., et al. Type I interferon-inducible gene expression in blood is present and reflects disease activity in dermatomyositis and polymyositis. Arthritis Rheum 2007, 56:3784-3792.
54. Ulfgren A.K., Grundtman C., Borg K., et al. Down-regulation of the aberrant expression of the inflammation mediator high mobility group box chromosomal protein 1 in muscle tissue of patients with polymyositis and dermatomyositis treated with corticosteroids. Arthritis Rheum 2004, 50:1586-1594.
55. Park J.H., Olsen N.J., King L., et al. Use of magnetic resonance imaging and P-31 magnetic resonance spectroscopy to detect and quantify muscle dysfunction in the amyopathic and myopathic variants of dermatomyositis. Arthritis Rheum 1995, 38:68-77.
56. Huh G.S., Boulanger L.M., Du H., et al. Functional requirement for class I MHC in CNS development and plasticity. Science 2000, 290:2155-2159.
57. Oliveira A.L., Thams S., Lidman O., et al. A role for MHC class I molecules in synaptic plasticity and regeneration of neurons after axotomy. Proc Natl Acad Sci U S A 2004, 101:17843-17848.
58. Fishman D., Elhyany S., Segal S. Non-immune functions of MHC class I glycoproteins in normal and malignant cells. Folia Biol (Praha) 2004, 50:35-42.
59. Engel A.G., Arahata K., Emslie-Smith A. Immune effector mechanisms in inflammatory myopathies. Res Publ Assoc Res Nerv Ment Dis 1990, 68:141-157.
60. Emslie-Smith A.M., Arahata K., Engel A.G. Major histocompatibility complex class I antigen expression, immunolocalization of interferon subtypes, and T cell-mediated cytotoxicity in myopathies. Hum Pathol 1989, 20:224-231.
61. Hohlfeld R., Engel A.G. HLA expression in myoblasts. Neurology 1991, 41:2015.
62. Nagaraju K., Raben N., Merritt G., et al. A variety of cytokines and immunologically relevant surface molecules are expressed by normal human skeletal muscle cells under proinflammatory stimuli. Clin Exp Immunol 1998, 113:407-414.
63. Karpati G., Pouliot Y., Carpenter S. Expression of immunoreactive major histocompatibility complex products in human skeletal muscles. Ann Neurol 1988, 23:64-72.
64. Englund P., Nennesmo I., Klareskog L., et al. Interleukin-1alpha expression in capillaries and major histocompatibility complex class I expression in type II muscle fibers from polymyositis and dermatomyositis patients: important pathogenic features independent of inflammatory cell clusters in muscle tissue. Arthritis Rheum 2002, 46:1044-1055.
65. Nagaraju K., Raben N., Loeffler L., et al. Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies. Proc Natl Acad Sci U S A 2000, 97:9209-9214.
66. Bartoccioni E., Gallucci S., Scuderi F., et al. MHC class I, MHC class II and intercellular adhesion molecule-1 (ICAM-1) expression in inflammatory myopathies. Clin Exp Immunol 1994, 95:166-172.
67. Nyberg P., Wikman A.L., Nennesmo I., et al. Increased expression of interleukin 1alpha and MHC class I in muscle tissue of patients with chronic, inactive polymyositis and dermatomyositis. J Rheumatol 2000, 27:940-948.
68. Pavlath G.K. Regulation of class I MHC expression in skeletal muscle: deleterious effect of aberrant expression on myogenesis. J Neuroimmunol 2002, 125:42-50.
69. Salomonsson S., Grundtman C., Zhang S.J., et al. Upregulation of MHC class I in transgenic mice results in reduced force-generating capacity in slow-twitch muscle. Muscle Nerve 2009, 39:674-682.
70. Li C.K., 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, 175:1030-1040.
71. Vattemi G., Engel W.K., McFerrin J., et al. Endoplasmic reticulum stress and unfolded protein response in inclusion body myositis muscle. Am J Pathol 2004, 164:1-7.
72. De Bleecker J.L., Engel A.G. Expression of cell adhesion molecules in inflammatory myopathies and Duchenne dystrophy. J Neuropathol Exp Neurol 1994, 53:369-376.
73. Nagaraju K., Raben N., Villalba M.L., et al. Costimulatory markers in muscle of patients with idiopathic inflammatory myopathies and in cultured muscle cells. Clin Immunol 1999, 92:161-169.
74. Chevrel G., Granet C., Miossec P. Contribution of tumour necrosis factor alpha and interleukin (IL) 1beta to IL6 production, NF-kappaB nuclear translocation, and class I MHC expression in muscle cells: in vitro regulation with specific cytokine inhibitors. Ann Rheum Dis 2005, 64:1257-1262.
75. Monici M.C., Aguennouz M., Mazzeo A., et al. Activation of nuclear factor-kappaB in inflammatory myopathies and Duchenne muscular dystrophy. Neurology 2003, 60:993-997.
76. Henriques-Pons A., Nagaraju K. Nonimmune mechanisms of muscle damage in myositis: role of the endoplasmic reticulum stress response and autophagy in the disease pathogenesis. Curr Opin Rheumatol 2009, 21:581-587.
77. Lavery G.G., Walker E.A., Turan N., et al. Deletion of hexose-6-phosphate dehydrogenase activates the unfolded protein response pathway and induces skeletal myopathy. J Biol Chem 2008, 283:8453-8461.
78. Li M., Dalakas M.C. Expression of human IAP-like protein in skeletal muscle: a possible explanation for the rare incidence of muscle fiber apoptosis in T-cell mediated inflammatory myopathies. J Neuroimmunol 2000, 106:1-5.
79. Nagaraju K., Casciola-Rosen L., Rosen A., et al. The inhibition of apoptosis in myositis and in normal muscle cells. J Immunol 2000, 164:5459-5465.
80. Le Fourn V., Gaplovska-Kysela K., Guhl B., et al. Basal autophagy is involved in the degradation of the ERAD component EDEM1. Cell Mol Life Sci 2009, 66:1434-1445.
81. Temiz P., Weihl C.C., Pestronk A. Inflammatory myopathies with mitochondrial pathology and protein aggregates. J Neurol Sci 2009, 278:25-29.
82. O'Leary M.F., Hood D.A. Denervation-induced oxidative stress and autophagy signaling in muscle. Autophagy 2009, 5:230-231.
83. Ramachandran N., Munteanu I., Wang P., et al. VMA21 deficiency causes an autophagic myopathy by compromising V-ATPase activity and lysosomal acidification. Cell 2009, 137:235-246.