Abstract
The (idiopathic) inflammatory myopathies are a heterogenous group of diseases, including dermatomyositis (DM), different forms of polymyositis (PM), and inclusionbody myositis (IBM) (reviewed in refs. 1–4). Clinically, DM is distinguished from PM and IBM by characteristic skin manifestations. A microscopic feature highly characteristic of DM is perifascicular atrophy, which is due to degeneration of muscle fibers at the periphery of muscle fascicles secondary to microvascular damage. Quantitative morphological analyses suggest that depletion of capillaries is one of the earliest changes in DM. Immunofluorescence studies revealed the deposition of complement in or around microvascular endothelium in a significant proportion of capillaries (5). These observations support the concept that an antibody- or immune-complex-mediated response against a vascular—endothelial component is a primary pathogenetic mechanism in DM.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Hohlfeld, R. and Engel, A. G. (1994) The immunobiology of muscle. Immunol. Today 15, 269–274.
Engel, A. G., Hohlfeld, R., and Banker, B. Q. (1994) The polymyositis and dermatomyositis syndromes, in Myology (Engel, A. G. and Franzini-Armstrong, C., eds.), McGraw-Hill, New York, pp. 1335–1383.
Dalakas, M. C. and Sivakumar, K. (1996) The immunopathologic and inflammatory differences between dermatomyositis, polymyositis and sporadic inclusion body myositis. Curr. Opin. Neurol. 9, 235–239.
Mantegazza, R., Bernasconi, P., Confalioneri, P., and Cornelio, D. (1997) Inflammatory myopathies and systematic disorders: a review of immunopathogenic mechanisms and clinical features. J. Neurol. 244, 277–287.
Mendell, J. R., Garcha, T. S., and Kissel, J. T. (1996) The immunopathogenic role of complement in human muscle disease. Curr. Opin. Neurol. 9, 226–234.
Arahata, K. and Engel, A. G. (1986) Monoclonal antibody analysis of mononuclear cells in myopathies III. Immunoelectron microscopy aspects of cell-mediated muscle fiber injury. Ann. Neurol. 19, 112–125.
Hohlfeld, R. and Engel, A. G. (1991) Coculture with autologous myotubes of cytotoxic T cells isolated from muscle in inflammatory myopathies. Ann. Neurol. 29, 498–507.
Hohlfeld, R., Engel, A. G., Ii, K., and Harper, M. C. (1991) Polymyositis mediated by T lymphocytes that express the γ/δ receptor. N. Engl. J. Med. 324, 877–881.
Dalakas, M. C. (1995) Immunopathogenesis of inflammatory myopathies. Ann. Neurol. 37, S74—S86.
Griggs, R. C., Askanas, V., DiMauro, S., Engel, A. G., Karpati, G., Mendell, J. R., et al. (1995) Inclusion body myositis and myopathies. Ann. Neurol. 38, 707–713.
Askanas, V. (1997) New developments in heredity inclusion body myopathies. Ann. Neurol. 41, 421–422.
Askanas, V., Engel, W. K., and Mirabella, M. (1994) Idiopathic inflammatory myopathies. Inclusion-body myositis, polymyositis, and dermatomyositis. Curr. Opin. Neurol. 7, 48–456.
Lundberg, I., Brengman, J. M., and Engel, A. G. (1995) Analysis of cytokine expression in muscle in inflammatory myopathies. Duchenne dystrophy and non-weak controls. J. Neuroimmunol. 63, 9–16.
Confalonieri, P., Bernasconi, P., Cornelio, F., and Mantegazza, R. (1997) Transforming growth factor-β1 in polymyositis and dermatomyositis correlates with fibrosis but not with cell infiltrate. J. Neuropathol. Exp. Neurol. 56, 479–484.
Lundberg, I., Ulfgren, A.-K., Nyberg, P., Andersson, U., and Klareskog, L. (1997) Cytokine production in muscle tissue of patients with idiopathic inflammatory myopathies. Arthritis Rheum. 40, 865–874.
Tews, D. S. and Goebel, H. H. (1996) Cytokine expression profile in idiopathic inflammatory myopathies. J. Neuropathol. Exp. Neurol. 55, 342–347.
Tateyama, M., Nagano, I., Yoshioka, M., Chida, K., Nakamura, S., and Itoyama, Y. (1997) Expression of tumor necrosis factor-a in muscles of polymyositis. J. Neurol. Sci. 146, 45–51.
De Bleecker, J. L. and Engel, A. G. (1994) Expression of cell adhesion molecules in inflammatory myopathies and Duchenne dystrophy. J. Neuropathol. Exp. Neurol. 53, 369–376.
Bartoccioni, E., Gallucci, S., Scuderi, F., Ricci, E., Servidei, S., Broccolini, A., et al. (1994) MHC class I, MHC class II and intercellular adhesion molecule-1 expression in inflammatory myopathies. Clin. Exp. Immunol. 95, 166–172.
Tews, D. S. and Goebel, H. H. (1995) Expression of cell adhesion molecules in inflammatory myopathies. J. Neuroimmunol. 59, 185–194.
Cid, M.-C., Grau, J.-M., Casademont, J., Tobías, E., Picazo, A., Coll-Vinent, B., et al. (1996) Leucocyte/endothelial cells adhesion receptors in muscle biopsies from patients with idiopathic inflammatory myopathies. Clin. Exp. Immunol. 104, 476–473.
Gallucci, S., Provenzano, C., Mazzarelli, P., Scuderi, F., and Bartoccioni, E. (1998) Myoblasts produce IL-6 in response to inflammatory stimuli. Int. Immunol. 10, 267–273.
Engel, A. G. and Arahata, K. (1984) Monoclonal antibody analysis of mononuclear cells in myopathies. II Phenotypes of autoinvasive cells in polymyositis and inclusion body myositis. Ann. Neurol. 16, 209–216.
De Bleecker, J. L. and Engel, A. G. (1995) Immunocytochemical study of CD45 T cell isoforms in inflammatory myopathies. Am. J. Pathol. 146, 1178–1187.
Iannone, F., Cauli, A., Yanni, G., Kingsley, G., Isenberg, D. A., Corrigall, V. M., et al. (1996) T lymphocyte immunophenotyping in polymyositis and dermatomyositis. Br. J. Rheumatol. 35, 839–845.
Bender, A., Ernst, N., Iglesias, A., Dornmair, K., Wekerle, H., and Hohlfeld, R. (1995) T cell receptor repertoire in polymyositis: clonal expansion of autoaggressive CD8 T cells. J. Exp. Med. 181, 1863–1868.
Mantegazza, R., Andreetta, F., Bernasconi, P., Baggi, F., Oksenberg, J. R., Simoncini, O., et al. (1993) Analysis of T cell receptor repertoire of muscle-infiltrating T lymphocytes in polymyositis. J. Clin. Invest. 91, 2880–2886.
O’ Hanlon, T. P., Dalakas, M. C., Plotz, P. H., and Miller, F. W. (1994) Predominant TCR-αβ variable and joining gene expression by muscle-infiltrating lymphocytes in the idiopathic inflammatory myopathies. J. Immunol. 152, 2569–2576.
Lindberg, C., Oldfors, A., and Tarkowski, A. (1994) Restricted use of T cell receptor V genes in endomysial infiltrates of patients with inflammatory myopathies. Eur. J. Immunol. 24, 2659–2663.
Pluschke, G., Rüegg, D., Hohlfeld, R., and Engel, A. G. (1992) Autoaggressive myocytotoxic T lymphocytes expressing an unusual γ/δ T cell receptor. J. Exp. Med. 176, 1785–1789.
Hohlfeld, R. and Engel, A. G. (1992) Expression of 65-kd heat shock proteins in the inflammatory myopathies. Ann. Neurol. 32, 821–823.
O’ Hanlon, T. P., Dalakas, M. C., Plotz, P. H., and Miller, F. W. (1994) The αβ T-cell receptor repertoire in inclusion body myositis: diverse patterns of gene expression by muscle-infiltrating lymphocytes. J. Autoimmun. 7, 321–333.
Fyhr, I.-M., Moslemi, A.-R., Tarkowski, A., Lindberg, C., and Oldfors, A. (1996) Limited T cell receptor V gene usage in inclusion body myositis. Scand. J. Immunol. 43, 109–114.
Fleischer, B. (1995) Superantigens. Acta Pathol. Microbiol. Immunol. Scand. (C) 102, 3–12.
Kotzin, B. L., Leung, D. Y. M., Kappler, J., and Marrack, P. (1995) Superantigens and their potential role in human disease. Adv. Immunol. 54, 99–166.
Karpati, G., Pouliot, Y., and Carpenter, S. (1988) Expression of immunoreactive major histocompatibility complex products in human skeletal muscles. Ann. Neurol. 23, 64–72.
Emslie-Smith, A. M., Arahata, K., and Engel, A. G. (1989) Major histocompatibility complex class I antigen expression, immunolocalization of interferon subtypes, and T cell-mediated cytotoxicity in myopathies. Hum. Pathol. 20, 224–231.
Bender, A., Behrens, L., Engel, A. G., and Hohlfeld, R. (1998) T-cell heterogeneity in muscle lesions of inclusion body myositis. J. Neuroimmunol. 84, 86–91.
Mikol, J. and Engel, A. G. (1994) Inclusion body myositis, in Myology. Basic and Clinical. (Engel, A. G. and Franzini-Armstrong, C., eds.), McGraw-Hill, New York, pp. 1384–1398.
Carpenter, S. (1996) Inclusion body myositis. J. Neuropathol. Exp. Neurol. 55, 1105–1114.
Pruitt, J. N., Showalter, C. J., and Engel, A. G. (1996) Sporadic inclusion body myositis: counts of different types of abnormal fibers. Ann. Neurol. 39, 139–143.
Garlepp, M. J., Laing, B., Zilko, P. J., Ollier, W., and Mastaglia, F. L. (1994) HLA associations with inclusion body myositis. Clin. Exp. Immunol. 98, 40–45.
Garlepp, M. J. and Mastaglia, F. L. (1996) Inclusion body myositis. J. Neurol. Neurosurg. Psychiatr. 60, 251–255.
Orimo, S., Koga, R., Nakamura, K., Arai, M., Tamaki, M., Sugita, H., et al. (1994) Immunohistochemical analysis of perforin and granzyme A in inflammatory myopathies. Neuromusc. Disord. 4, 219–226.
Goebels, N., Michaelis, D., Engelhardt, M., Huber, S., Bender, A., Pongratz, D., et al. (1996) Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis. J. Clin. Invest. 97, 2905–2910.
Cherin, P., Herson, S., Crevon, M. C., Hauw, J.-J., Cervera, P., Galanaud, P., et al. (1996) Mechanisms of lysis by activated cytotoxic cells expressing perforin and granzyme-b genes and the protein TIA-I in muscle biopsies of myositis. J. Rheumatol. 23, 1135–1142.
Liu, C.-C., Walsh, C. M., and Young, J. D. E. (1995) Perforin: Structure and Function. Immunol. Today 16, 194–201.
Henkart, P. A. (1994) Two pathways and multiple effector molecules. Immunity 1, 343–346.
Behrens, L., Bender, A., Johnson, M. A., and Hohlfeld, R. (1997) Cytotoxic mechanisms in inflammatory myopathies. Co-expression of Fas and protective Bc1–2 in muscle fibers and inflammatory cells. Brain 120, 929–938.
Schneider, C., Gold, R., Dalakas, M. C., Schmied, M., Lassmann, H., Toyka, K. V., et al. (1996) MHC class I mediated cytotoxicity does not induce apoptosis in muscle fibers nor in inflammatory CT cells: studies in patients with inclusion body myositis. J. Neuropathol. Exp. Neural. 55, 1205–1209.
Inukai, A., Kobayashi, Y., Ito, K., Doyu, M., Takano, A., Honda, H., et al. (1997) Expression of Fas antigen is not associated with apoptosis in human myopathies. Muscle Nerve 20, 702–709.
Tews, D. S. and Goebel, H. H. (1997) Apoptosis-related proteins in skeletal muscle fibers of spinal muscular atrophy. J. Neuropathol. Exp. Neural. 56, 150–156.
Lee, R. K., Spielman, J., and Podack, E. R. (1996) bcl-2 protects against Fas-based but not perforin-based T cell-mediated cytolysis. Int. Immunol. 8, 991–1000.
Farrow, S. N. and Brown, R. (1996) New members of the Bc1–2 Family and their protein partners. Curr. Opin. Genet. Dev. 6, 45–49.
Lynch, D. H., Ramsdell, F., and Alderson, M. R. (1995) Fas and FasL in the homeostatic regulation of immune responses. Immunol. Today 16, 569–574.
Brunner, T., Mogil, R. J., LaFace, D., Yoo, N. J., Mahboubi, A., Echeverri, F., et al. (1995) Cell-autonomous Fas (CD95)/Fas-ligand interaction mediates activation-induced apoptosis in T-cell hybridomas. Nature 373, 441–444.
Goebels, N., Michaelis, D., Wekerle, H., and Hohlfeld, R. (1992) Human myoblasts as antigen presenting cells. J. Immunol. 149, 661–667.
Hohlfeld, R. and Engel, A. G. (1990) Induction of HLA-DR expression on human myoblasts with interferon-gamma. Am. J. Pathol. 136, 503–508.
Mantegazza, R., Hughes, S. M., Mitchell, D., Travis, M., Blau, H. M., and Steinman, L. (1991) Modulation of MHC class II antigen expression in human myoblasts after treatment with IFN-y. Neurology 41, 1 128–1132.
Hohlfeld, R. and Engel, A. G. (1990) Lysis of myotubes by alloreactive cytotoxic T cells and natural killer cells. Relevance to myoblast transplantation. J. Clin. Invest. 86, 370–374.
Lindberg, C., Oldfors, A., and Tarkowski, A. (1995) Local T-cell proliferation and differentiation in inflammatory myopathies. Scand. J. Immunol. 41, 421–426.
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2000 Springer Science+Business Media New York
About this chapter
Cite this chapter
Goebels, N., Hohlfeld, R. (2000). Inflammatory Myopathies. In: Tsokos, G.C. (eds) Principles of Molecular Rheumatology. Current Molecular Medicine, vol 1. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-018-6_22
Download citation
DOI: https://doi.org/10.1007/978-1-59259-018-6_22
Publisher Name: Humana Press, Totowa, NJ
Print ISBN: 978-1-61737-182-0
Online ISBN: 978-1-59259-018-6
eBook Packages: Springer Book Archive