IDIOPATHIC INFLAMMATORY MYOPATHIES – RECENT DEVELOPMENTS Edited by Jan Tore Gran Idiopathic Inflammatory Myopathies – Recent Developments Edited by Jan Tore Gran Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access articles distributed under the Creative Commons Non Commercial Share Alike Attribution 3.0 license, which permits to copy, distribute, transmit, and adapt the work in any medium, so long as the original work is properly cited. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published articles. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book. Publishing Process Manager Alenka Urbancic Technical Editor Teodora Smiljanic Cover Designer Jan Hyrat Image Copyright 3DO!, 2011. Used under license from Shutterstock.com First published September, 2011 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org Idiopathic Inflammatory Myopathies – Recent Developments, Edited by Jan Tore Gran p. cm. ISBN 978-953-307-694-2 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Preface IX Chapter 1 Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis 1 Cristina Cappelletti, Lucia Morandi, Marina Mora, Franco Salerno, Paolo Confalonieri, Renato Mantegazza and Pia Bernasconi Chapter 2 Serological Aspects of Myositis 25 Johannes Schulte-Pelkum Chapter 3 Arthrogenic Alphaviruses and Inflammatory Myopathies 43 Lara Herrero and Suresh Mahalingam Chapter 4 The Antisynthetase Syndrome 65 Jan Tore Gran and Øyvind Molberg Chapter 5 Aminoacyl-tRNA Synthetases in Idiopathic Inflammatory Myopathies: An Update on Immunopathogenic Significance, Clinical and Therapeutic Implications 77 Codrina Mihaela Ancuta, Eugen Ancuta and Rodica Marieta Chirieac Chapter 6 Interstitial Lung Disease Associated with Clinically Amyopathic Dermatomyositis 91 Toshinori Takada, Eiichi Suzuki and Ichiei Narita Chapter 7 Myositis and Cancer 111 Sergio Prieto-González, Vladimir Lazo, Ernesto Trallero-Araguás, Albert Selva-O’Callaghan and Josep M Grau Chapter 8 Orbital Myositis 123 Toshinobu Kubota VI Contents Chapter 9 Myouclear Breakdown in Sporadic Inclusion Body Myositis 143 Satoshi Nakano and Hirofumi Kusaka Chapter 10 Immunoglobulin Treatment in Polymyositis and Dermatomyositis 163 Maria Giovanna Danieli, Lucia Pettinari, Romina Moretti, Francesco Logullo and Armando Gabrielli Chapter 11 Pregnancy in Myositis: Challenges and Pitfalls 197 Codrina Mihaela Ancuta, Eugen Ancuta and Rodica Marieta Chirieac Preface The idiopathic inflammatory myopathies present some of the most challenging clinical problems in medicine. For many years, these myopathies were simply classified into polymyositis, dermatomyositis and myositis associated with other connective tissue diseases. The treatment relied solely on administration of glucocorticosteroids. Fortunately, our understanding of the immunology, pathology, genetics and clinical aspects of the idiopathic inflammatory myopathies has increased enormously in recent years. The discovery of the myositis specific autoantibodies is of particular imporance, linking specific immunological reactions to distinct clinical subsets of myositis. These advances will most likely contribute to improved definitions and classification of the myopathies, and in due time better treatment and care for the patients. The clinicians involved will be equipped with better tools for predicting disease outcome and final prognosis. As new important findings are continously published, reliable and up-to-date reviews are strongly warranted. The emphasis of this book is to provide the reader with recent developments in etiopathogenesis, diagnostics and treatment of the idiopathic inflammatory myopathies. The purpose has not been to present comprehensive descriptions of all myopathic disorders, which can be found in most traditional textbooks. Our goal was to have chapters written by authors with the most expertice. The contributors to this book represent some of the most competent and dedicated workers in the field of myopathies today. I am confident that the book will provide physicians of varied medical specialities with new information, which will be of great value to their research and patient care. Jan Tore Gran Consultant, Professor dr. med. Departement of Rheumatology Oslo University Hospital, Rikshospitalet Sognsvannsveien 200026, Oslo Norway [...]... 07554982 18 Idiopathic Inflammatory Myopathies – Recent Developments Dalakas, M.C (2011c) Review: an update on inflammatory and autoimmune myopathies Neuropathology and Applied Neurobiology, Vol 37, No 3, (Apr., 2011), pp (226-242), ISSN: 0305-1846 De Paepe, B.; De Keyzer, K.; Martin, J.J & De Bleecker, J.L (2005) Alpha-chemokine receptors CXCR1-3 and their ligands in idiopathic inflammatory myopathies. .. plasma cells – that have undergone affinity maturation and switched their isotype from IgM to IgG or IgA – are abundant in PM muscle (Greenberg, 2007a; Salajegheh et al., 2010b; Cappelletti et al., 2011) In view of the recently demonstrated ability of TLR9 to induce B cells to mature to plasma cell in vitro (Giordani et al., 2009), together with the 12 Idiopathic Inflammatory Myopathies – Recent Developments. .. in muscle of patients with idiopathic inflammatory myopathies and cultured muscle cells Clinical Immunology, Vol 92, No 2, (Aug., 1999), pp (161-169), ISSN: 15216616 Nagaraju, K.; Rider, L.G.; Fan, C.; Chen, Y.W.; Mitsak, M.; Rawat, R.; Patterson, K.; Grundtman, C.; Miller, F.W.; Plotz, P.H.; Hoffman, E & Lundberg, I.E (2006) 22 Idiopathic Inflammatory Myopathies – Recent Developments Endothelial cell... and other idiopathic inflammatory myopathies of childhood The Lancet, Vol 371, No 9631, (Jun., 2008), pp (2201-2212), ISSN: 0140-6736 Idiopathic Inflammatory Myopathies: A Review on Immunopathological Features and Current Models of the Pathogenesis 19 Figarella-Branger, D.; Civatte, M.; Bartoli, C & Pellissier, J.F (2003) Cytokines, chemokines, and cell adhesion molecole in inflammatory myopathies. .. Role of cytokines and chemokines in idiopathic inflammatory myopathies Current Opinion in Rheumatology, Vol 21, No 6, (Nov., 2009), pp (610-616), ISSN: 1040-8711 Dimachkie, M.M (2011) Idiopathic inflammatory myopathies Journal of Neuroimmunology, Vol 231, No 1-2, (Nov., 2010), pp (32-42), ISSN: 0165-5728 Distad, B.J.; Amato, A.A & Weiss, M.D (2011) Inflammatory myopathies Current Treatment Options... associated protein-5 (MDA-5), one of the RIG-I-like receptors involved in the 4 Idiopathic Inflammatory Myopathies – Recent Developments recognition of viral RNAs during the innate immune responses RIG-I and IFIH1 interact with viral RNA and mediate signalling pathways leading to the transcription of type I interferons (IFNs) and inflammatory cytokines (Takeuchi & Akira, 2008) The finding that IFIH1 provokes... exposures in etiology Arthritis & Rheumatism, Vol 56, No 8, (Aug., 2007), pp (271 9–2 728), ISSN: 0004-3591 Walsh, R.J.; Kong, S.W.; Yao, Y.; Jallal, B.; Kiener, P.A.; Pinkus, J.L.; Beggs, A.H.; Amato, A.A & Greenberg, S.A (2007) Type I interferon-inducible gene expression in blood 24 Idiopathic Inflammatory Myopathies – Recent Developments is present and reflects disease activity in dermatomyositis and polymyositis... for this receptor or its involvement in the regeneration or differentiation of damaged muscle tissue Over-expression of type I IFNs might be involved in muscle fiber atrophy 14 Idiopathic Inflammatory Myopathies – Recent Developments Fig 5 Proposed immunopathogenetic mechanisms in PM In PM upregulation of type I and type II IFNs, possibly caused by a pathogenic agent or by intracellular autoantigens... signature in the peripheral blood of dermatomyositis patients is associated with disease activity Molecular Medicine, Vol 13, No 1-2, (Jan-Feb., 2007), pp (59-68), ISSN: 10761551 16 Idiopathic Inflammatory Myopathies – Recent Developments Basta, M & Dalakas, M.C (1994) High-dose intravenous immunoglobulin exerts its beneficial effect in patients with dermatomyositis by blocking endomysial deposition of... and treatment of inflammatory myopathies Current Rheumatology Reports, Vol 9, No 4, (Aug., 2007), pp (291-297), ISSN: 1523-3774 Dalakas, M.C (2011a) Immunotherapy of inflammatory myopathies: practical approach and future prospects Current Treatment Options in Neurology, Published online (Mar., 2011), ISSN: 1092-8480 Dalakas, M.C (2011b) Pathophysiology of inflammatory and autoimmune myopathies Presse . IDIOPATHIC INFLAMMATORY MYOPATHIES – RECENT DEVELOPMENTS Edited by Jan Tore Gran Idiopathic Inflammatory Myopathies – Recent Developments Edited. Additional hard copies can be obtained from orders@intechweb.org Idiopathic Inflammatory Myopathies – Recent Developments, Edited by Jan Tore Gran p. cm. ISBN 978-953-307-694-2 free. ultraviolet light (UVA or UVB) (Hengstman et al., 2000; Love et al., 2009). This Idiopathic Inflammatory Myopathies – Recent Developments 2 photosensitivity appears associated with the TNF-α-308A